Case: Child with KD who developed macrophage activation syndrome (MAS), a life-threatening complication. Features included falling counts, falling platelets, thrombocytopenia, high TRIG, low fibrinogen, and bad fevers. Children with MAS can die within 24 hours. This demonstrates KD can cause the severest life-threatening complications.
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Hello, good evening all I Dr. Kundan Chopra EB member of Maharashtra. On behalf of MAYIP, welcome you all to yet another episode of subsp specialty spotlight. This is the fifth episode in sub specialy spotlight. After successful webinar on cardiology, endocrinology, hematology and neurology.
Today's spotlight is on rheumatology. I welcome all office bearsers of MAIP, EB members of central IIP and MAIP. And today's expert none other than father figure in pediatric rheumatology Dr. Dr Rajuk Jandani sir and today's speaker senior most pediatric rheatologist Dr. Girishi Subramanyam sir Dr. Shikhaal ma'am from Mumbai and Dr. Himi Chhri ma'am from Pune Dr. Kundan Chopra along with Dr. Bupes Bon our moderator for today's webinar on this Mahip platform virtual platform and advisor for this sub specialy spotlights are Dr. Nikil Paddak and Dr. Raindra Sononyi we will start the webinar. Uh I would like to ask our uh president sir uh sir has joined ma'am I think sir is traveling he has conveyed me his regards. Uh so Dr. Reu Borak ma'am for her expert comment ma'am.
>> Yeah good evening one and all. It is a very honor for my platform to have the father of pediatric rheumatology and a national and international figure in rheumatology Dr. Kop Chandan sir and he is a teacher of teacher and I have been uh for the promise course of pediatric rheumatology done by him online in September 2024. So I'm very much happy to have sir on this platform. Welcome sir and also uh the speakers of today senior pediatric rheumatologist Dr. Girish Subramanyan sir Dr. Sika Graal my dear friend from Mumbai and Dr. Himmani Chowrii uh all are welcome to this platform and they will be giving us the insights of periodic rheumatology and after doing the promise course I really can say that it is like sir was like a Sherlock Holmes and we used to do the puzzles and the jigsaw puzzle sir used to show the inflammatory part and this thing it was that time I got into rheumatology and whenever there is a topic of rheumatology I'm very much keen attending it >> so Welcome to this platform all of you.
I also welcome all the dignitaries of CIP Mahip also the scientific convenor and the chairperson Dr. Ba man and the team and today's MOC who has taken all the efforts for arranging this program uh right from the beginning Dr. Kundan Chopre as well as Drs. Bopees bond uh I would like to congratulate them for a successful uh organization and in absentia our president Dr. Sanjiv Zoshi uh I would like to welcome you all on his behalf as he's traveling he will be joining around 9:30 p.m. I think so. So I would like uh Kundan to hand over to Dr. Bailaw ma'am for a few words of wisdom and then you can take it off.
Thank you.
>> Yes Dr. Bor ma'am.
>> Yeah. Good evening everyone and uh as always I would still like to congratulate the team of my for bringing together such stalwarts today for such important topics. It's going to be great learning and a pleasure to hear Chandani sir Sikha and Himmani Chri and Sugramanyam sir on important topics which are very important for our daily office practice. to uh thank you for arranging this wonderful sub specialtity uh CME today.
>> Thank you ma'am. Thank you. Uh Dr. Yogesh Parik sir has also joined sir sir Karishma is that kungchani sir that not only from Maharashtra from other states also have joined for today's this webinar from him Dr. Yogi sir can you unmute?
>> Yeah thank you dear Dr. Reenu madam and Dr. Kundan and Da madame Dr. Kumadani Dr. Shikhard Dr. Girish and all. So best wishes for the great successful program because I am learning so much from the Mahayappi also now. So I'm regularly attending the program whenever the it required. Thank you uh Dr. Renu madam and Dr. Kundan. Over to you please.
>> Thank you. Thank you.
>> Thank you sir.
>> Yeah Dr. Sagar Balasar is there also.
Secretary admin sir your >> good evening and welcome sir Dr. Girish sir Dr. Shika madam and Dr. Human Chri madam and all the best my pen master class.
>> Thank you. Yes I will give you overview of today's program. First lecture will be by Dr. Girish Subramanyam senior most rheumatologist from Nagpur. He will talk on role of ANA in pediatric rheumatology. Second talk will be by Dr. Shikhaal ma'am from Mumbai. She will give about an approach to child with joint swelling in OPD. And third talk is by Himi Chhri ma'am on Kawasaki disases the complete the incomplete and the typical. After each talk our esteem expert will give his expert comment and uh all the attendees can ask their question in their chat box and all questions will be taken at the end of all three talks. Uh respective expert will uh expect speaker will give and expert opinion also given on our questions. So uh at the start only I will just introduce our all speakers. uh ma'am uh speaker the first important I will uh give introduction to Dr. Rajukuman Gupt Chandani sir uh sir I think needs no introduction sir has given only one slide of introduction Dr. Rajup Chandani sir consultantist and institution name SRCC children's hospital uh second speaker is Dr. Gish first speaker is Dr. Dr. Girish Subraman sir he consultant pediatrician and pediatric rheumatologist from Nagpur.
Sir has more than 25 years of expert in pediatrics and more than 10 years of expertise in pediatric rheatology. Our third uh second talk will be given by Dr. Shikhaal ma'am. She is pediatric rheumatologist and consultant founder of pediatric rheatology and expert practice clinic. Then she also uh visits at Nanawati Max super specialy hospital also at Surya PD Hinduja and BJI hospital in Mumbai. She has multiple papers in index journal and book chapters in his uh books and she is secretary of IIP Mumbai. She is EB member of pediatric rheatology society in 2023 and 24 and Dr. Dr. Himmani Chowri is from done MD pediatric and DM pediatric clinical iminology and rheumatology from Piji Chandigar and also she has ESID short-term fellowship in clinical imunology at Rottenham Rottenham Nether Netherland. She had specialist training in blood and marot transansfusion and cellular therapy from Tata Memorial Hospital and Grand Orman Street Hospital London. She is a consultant clinical iminology and pediatric rheumatology uh at Ibrahan Clinic Pune and she is also associated with Rubial Hall clinic Jagir hospital and Dinat Mangeshkar hospital Pune. Uh she will be talking on Kawasaki diseases. Uh without wasting much topic I will hand over the mic and all uh uh PPT to Dr. Subraman sir who will start with first lecture that is role of AN pediatric rheumatology. Sir you can share your slides. Slides are visible sir.
G sir sir please start sir. Audible sir please start. Thank you.
>> Let us begin today's discussion anti-uclear antibodies with a couple of cases that we saw in the recent past.
Both cases were very similar.
Studies are not moving. The slides are not moving.
Double click the on that screen. Yes.
Yes. Thank you, sir.
>> This was patient that we saw just a couple of days ago. This 10-year-old boy presented with fever of one month duration had organomedally significant hepto and cytoine.
The treatian initially thought that the patient had leukemia and got a bone marrow die. The bone marrow came back as normal and then the patient was referred to me on detailed head to2 examination.
I realized that the patient had palat erosions and I felt that it could be got an ANA done which was 1 in 160 positive.
Subsequently further tests for specific antibodies and DSDNA and antismith were done which also turned out to be positive. The final diagnosis of SL was made. A similar case that we encountered a month ago was a 12-year-old female who also presented a fear of almost a month's duration of spending cytoineas and also had arthritis.
We were quite certain that this will be a sel and quite correctly our title came back at morning 640.
Fortunately for us on our rounds we realized that the patient was in significant pain because of arthritis.
And when we asked a detailed history the mother said that she's unable to sleep at night on many nights because of the pain. That start that made us start thinking whether it could be leukemia because it's a very important rule in pediatric rheatology that if the bone or the joint pain disturb the child at night think of leukemia. We got a bone marrow done and that patient turned out to have leukemia.
So two similar cases both seemingly very typical of SLE both having ANA positive too but one turned out to have leukemia the other turned out to these two cases illustrate that ANA can be a great ally great friend but at the same time it can be great distractor it can distract you away from the diagnosis so now I take a series of questions frequently asked questions by general pediatricians about ANA and discuss each question in detail. When should you order an ANA?
When you should you should not order is for non-specific symptoms like a child coming with nonspecific arthalgia, low-grade fever, fatigue, do not order an ANA. Order an ANA only if the clinical signs and symptoms suggest an autoimmune systemic rheumatic disease.
like SL, join, stler, juvenile dermatitis in mix connected tissue disorder and the list of conditions which are included under ANA positive rheumatic diseases in children.
Pre-est probability that is the likelihood that the patient has the disease before you run the test is of utmost importance when it comes to ordering an ANA. This is because 20 to 30% of children will have a positive ANA in no titers especially in no titers and if you are not aware of this and if you do a ANA without a preest probability then you are stepping on a banana peel.
If you are reasonably certain before you order the test that this patient has a very high likelihood of of a pediatric rheumatological disorder then ANA will be of great benefit to you.
The ANA is a highly sensitive test but poorly specific.
It is gets more and more specific as the title goes up less becomes less and less specific as the title goes down.
Some clinical examples of where you would order an ANA because the pre-est probability is high is an adolescent girl who has photosensitive rash and plural fusion. An adolescent girl who has reord phenomenon with skin changes.
A child with unexplained dry eyes, dry mouth and recurrent paratitis and a child and an adolescent girl again with significant alopeesia and systemic symptoms. So these four examples illustrate that in the where situations where the pre-est probability of a positive ANA ANA is very high and such situations will help you make sure that you do not land up with false positives.
This is a frequently asked question.
Which titer of ANA is considered positive? The simple answer is there is no titer which is taken as definitely positive or definitely negative. As the titer goes up, the chances of this being a rheumatological disorder is very high.
And the titer keeps going down the chances of this being rheumatological disorder goes down. But there is no specific titer at which you can say that this is definite ANA positive or this is definite ANA negative. Having said that a titer of 1 in 40 or 1 in 80 is generally not significant. A titer of 1 in 160 is taken as significant and a titer of 130 and higher strongly suggests an underlying immune disease autoimmune disease.
So remember ANA is a marker of probability which is not a marker of severity. High title only confirms that the patient is immune system is highly reactive against nuclear antigens. It diagnosis still requires good clinical acumen and good diagnostic skills.
Are there any non-rumatological conditions which are associated with NA positivity? That's a long list. You can have NA positivity with infections. No wonder children have such a high percentage of false positive ANA. So ANA can be positive in many infections some of which I have listed here. Can be positive in organ specific autoimmune diseases like Hashimotoyroiditis, primary beterosis, autoimmune hepatitis, malignancies and we saw the first example that I discussed child with leukemia had a significantly high ANA titer.
Malignancies can lead to ANA positivity medications. We're all aware that many medications can lead to false positive ANA. Patient who is on TNF inhibitors can have positive positive ANA without having any symptoms of any rheumatological disorder. And the most important 15 to 20% of completely healthy children and elderly persons and pregnant women can have a positive ANA.
Apart from this, you must remember that not all rheumatological disorders have a positive ANA. For example, vasculitis disorders and uh most cases of ja you will not have a positive.
The best screening method screening method is by indirect imoflloresence.
Eliza is far inferior to indirect imoflloresence.
Indirect imoflloresence is done on Hepu cells. Hepu stands for human epithel carcinoma cells.
This hipto cells are cells which have very large nucleus nucleus and a large cytoplasm and they exhibit a huge number of antigens. Among those number of antigens are expressed by these hepto cells.
So many antigens cannot be captured or by the eliza method. So eliza the number of antigens is much lower than that which is seen with indirect imlloresence. Hence it suffers in comparison and many times eliza when it detects a negative ANA you'll find it turns positive when you do it by indirect imlorence and if you look at the figure here you'll find number of nuclear antigens which have been labeled there plus you'll find that there are many antigens in the mito cytoplasm also so indirect imoflloresence not only detects nuclear antigens it also detects antibodies two cytoplasmic antigens. Not only that being carcinoma cells they are actively multiplying and you will have many times cells in mito motic phase and motic antigens are picked up by indirect imunosence. So imoflloresence picks up antibodies such as nuclear nuclear antigens antibodies against cytoplasmic antigens antibodies against mrotic division antigens. So in reality it is not anti-uclear antibody it is anti-ell antibody but the term anti-uclear antibody is still retained because of it long use what is the importance of fluoresence which is reported in the ANA report depending on where the antigens are located the floresence will vary the DSDNA is located throughout the nucleus so you have a homogeneous pattern of fluoresence and it corresponds well with systemic lucas arithmettosis.
Speckled. Speckled is nuclear antigens which are not distributed so homogeneously but distributed all over the cell all over the nucleus but not so homogeneously and it is seen with number of antigens SSA, SSB, Smith ribboucle protein and hence it is less specific rather the least specific pattern is speckled.
Anti-entromeir is very specific for limited cutaneous systemic sclerosis formerly known as crest syndrome.
Anti-entromeir antibodies here the fluoresence is located on the syndrome here. Anticenter antibodies are very diagnostic of this condition.
Nuclear nuclear fluoresence is seen more often with diffuse systemic sclerosis.
Now you might wonder that once you have this staining pattern is it is it necessary to do specific antibbody test for example you have a homogeneous pattern on imunofllororesence should you go still go ahead with the DSDNA the answer is yes staining pattern is just a compass it is not the final arbittor so you have it can be it can misread you at many times for example a speckled pattern I've specifically told you there are so many antigens which can be which will show speckled pattern or imofense that it will be difficult to know which antibody is present so you have to do further antibody testing even if the staining pattern is very characteristic so it is suggestive not definitive exception being a centromeir pattern another exception is diffuse uh DFS diffuse fine speckled antibodies diffuse fine speckled antibodies if you see this antibodies in high tighter you are certain that this patient does not have a rheumatological disorder this is one antibody which is seen in very high tighter in patients who do not have rheumatological disorders it's a classic example of a false positive ANA never rely solely on a pattern to deduce the type of antibody you must follow up a positive ANA with specific antigen testing.
So after the after you obtain the ANA report when should you order further antibody testing? There are a few examples to illustrate when you should do it.
So the testing is based on clinical symptoms. If you feel that the clinical symptoms are highly suggestive of SLE then you should go ahead with just DSDNA and antismith. If the patient clinical profile suggest syndrome then you should do anti- row and antila.
If the ANA test is positive in a pregnant female do antibodies against row and LA.
And finally, if the ANA test is negative and your clinical suspicion of SL is very high, then you must test specific antibodies against row 60, row 52 and ribosomeal P. These antibodies are not detected definitely by HEP2 based imloros. These are classic examples of false negative ANU.
So if your client suspicion of SL is very high extremely high and the ANA is negative first make sure that you still stick to the diagnosis revise revisit your diagnosis and still if you feel it is SLE then you must do specific antibbody testing for anti- row and anti ribosomal by Eliza method.
Some of you might wonder why should I break my head on which antibody to be tested? Why not use a multi- antigen strip what is known as an ANA blot or ENA profile rather than testing individual antibodies. For one, it saves you a lot of money. You do everything in one go. And for number two, everything is available to you on a platter. But this is highly felacious.
often times for a you do this for a patient who has a very classical picture of SL and you land up with certain antibodies it just doesn't correlate with the clinical picture and you're in a big soup so do those antibodies do ENA in specific situations where there are overlapping features of two rheumatological disases then you do this so if you have a high title ANA ANA the patient with overlapping features For example, a 12-year-old female with malarian arthritis and proteinura. Plus, this patient also has recurrent paratritis. So, you know that there are overlapping features between SLE and joining syndrome. Individual antibodies will test when you suspect one specific disease. In such a situation, do not go with the ANA ENA. And very important, never order an ANA block for a healthy child who has an incidental low tighter ANA.
Can ANA give you some idea of prognosis?
Can be can it be used as a monitoring tool? Number one, it has absolutely no prognostic tool role and ANA once positive remains positive forever. Do not think that having treated the patient the ANA should become negative. That is number one.
Don't think that the titer should go down if the treatment have been perfect.
That does not occur as a prognostic marker.
DNA is of no use as a monitoring tool.
It is of no use but both as a prognostic as and a monitoring tool antidsDNA is of great benefit. If you have a patient of SLE freshly diagnosed patient and DSDNA is very high and there's a high risk that this patient will develop lupus nephritis and if you are treating a patient of lupus nephritis serial titus of antidsna will tell you whether the patient is improving or not improving apart from other parameters of course.
So I told you that a tighter of one in 160 is taken as significant. A tighter of 1 in 320 is more significant. Ter of 1 in 80 is usually not significant at all. So many of you might argue that the recent the latest 2019 UNR ACR criteria takes one in 80 as a entry criterion.
Why is this so? These criteria are designed to be very sensitive and very specific both at the same time. By lowering the entry criterion to one in 80 the criteria have become very very sensitive but not at the cost of specificity because they have then added imunological and clinical domains and the domains have been added in such a way the scoring system has been framed in such a way that at the end the criteria become very specific. So both sensitivity and specificity is maintained in the UR criteria. Hence 1 in 80 is taken as entry criteria. But by and large in your clinical day-to-day practice, you should take one in 80 title with a pinch of salt. One in 160 and above is significant.
I hope I finished with your time. Thank you very much for >> Yes sir. Thank you sir. It was excellent talk. Uh Buchchani sir your expert comment on this.
>> Yes. So Girish as usual was completely on the ball.
I would just like to reemphasize three of his very important messages.
The first message he tried to state was do not let the tail wag the dog. Ask for this test only if you are suspecting something specific.
Do not keep repeating the test. It has no value in terms of following up the disease. And don't ask for the auto antibbody profile at the same time.
First, wait to see what the ANA shows you.
Now, however, there are a couple of additional points that I might like to make.
The first being that the ANA is also an important test in the oligo articular juvenile idiopathic arthritis because there it is a stratification test not to diagnose the condition but to identify the risk for uvitis. And according to the American Academy of Opthalmology, those typical 2 to 5 year old females who have oligoicular disease and who are ANA positive need more frequent eye checkups as compared to the other ones who are not ANA positive.
The second point peculiarly is the problem that we face when we are practicing pediatric rheumatology.
One overenthusiastic pediatrician would have done an AMA against what Girish has taught us today.
And this title comes back as 1 in 1280 or 1 in 2560 and the child is dancing about. What do you do at this stage? Because you are stuck with a report which was done on some grounds by the primary pediatrician. But now you are holding the hot potato because you have to explain to the parents how to interpret this report.
So there was one small omission by Girish and that is that if you have a relatively asymptomatic ANA positive child, please screen this child for uvitis.
We had a child exactly like this just last week in our office and we screened for uvitis. Uvitis was negative. Clinical exam was normal.
We tested thyroid just to look for subclinical hypothyroidism negative liver function test because these are the ones that could be silent.
So LFT normal, thyroid normal, celiac screen negative.
So all that we've done is told the child please go home nothing to be done just follow up for prolonged fever recurrent paratritis uh rashes on the face mouth ulcers re not phenomenon. So go through the list of connective tissue diseases and as far as lupus is concerned to see a negative lupus I have not seen any so far. It may have been mentioned in textbooks, but if you see a negative ANA, by all considerations, you could consider maybe 99.5% that you have ruled out lupus. That's all I would have to say.
>> Thank you, sir.
>> So, we will move to our next lecture. Uh Dr. Dr. Shikharal will talk an approach to child with joint swelling in OPD.
Over to you ma'am.
>> Thank you so much. Thank you team I Mahip for giving this opportunity. Uh I'll just share my screen.
Yes, slides are visible. Make it full screen now.
>> Yeah, >> perfect.
Okay. So, after a very wonderful presentation on ANA by Dr. Girish Sur, we move on to a very simple topic of joint swelling. When a child presents with a joint swelling in our OPD, OPD.
So, first let's get into the mode of a joint. I just wanted to reiterate a fact that there is joint which is the joint capsule the cartilage the joint space and the sinovial membrane and there are the perioticular tissues which also can present like a joint swelling. So the muscle inflammation a berscitis a tendinitis or for that sake anthsitis where the tendons are inserted on the bone if they get inflamed then they can present like a joint swelling. So what do we call when when do we call or define that the child is having arthritis? Sometimes when the child is having just pain then the parents may come and tell you that but arthritis but just pain doesn't define arthritis it is arthralgia. Along with that if you have signs of inflammation like heat or warmth over the joint or if there is a swelling of the joint or if there is a limitation of joint movement that is when we define that the child will be having arthritis. So coming when we are seeing a child in the OPD basis we always need to take very a good history when we are seeing a child with a joint swelling and do a good examination because our 90% of the diagnosis of a child will depend on these two things a good history and examination onset progression duration is something we ask to all children when they present with any symptom. So what is the onset?
Whether we are seeing an acute onset which is less than 6 weeks of duration or we are seeing a chronic onset which is usually divi defined as more than 6 weeks duration of a joint swelling whether the swelling appeared suddenly like an acute setting or if it's a gradual. Another very important and pertinent history to ask in any child who is presenting with joint swelling is the timing of joint pain. So if the child if the parents say that early morning stiffness is there or morning pain is there then we know that we may be dealing with something which is inflammatory in nature evening by the evening if there is a joint pain which is not associated with swelling lot of times it's a mechanical pain so growing pains can also present with evening pains and night pains we need to be very cautious when the parents say that the child is sick and gets up at night with joint pain or bone pain at night then that history we need to see that there are sinister findings we need to look for when the child presents or parents give a history. So timing of pain of joint is important. Whether there is a limp associated abnormal gate and another important thing the look of the child whenever the child enters in our clinic we always see whether the child is a very sick-looking child or a child is otherwise a well-looking child just presenting with single joint or a two joint swelling or pain of that joint because that will help us to lead to a differential diagnosis. also ask relevant questions trauma but we need to figure out whether it's just a trivial trauma because if we ask any mother but they always say yes but we need to decipher whether it's just a trivial trauma or it's a relevant trauma which has led to that joint swelling or pain associated systemic findings of fe fever fatigue weight loss appetite loss eye redness is there or not because in certain cases acute anterior uvitis or conjunctivitis In reactive arthritis may be associated with eye redness. We should also ask a history of rash ulcer nodules, a history of past history of viral illness and do not forget if a small boy is presenting with history of joint swelling. We should ask history of any bleeding diiathesis in the family as well.
Coming to the examination, so we to exclude we do the examination. A good proper PALS examination for any child who is presenting any clumsy child or child presenting with a muscularkeeletal symptom is very important to do a pediatric gate arm leg spine. We look feel and move the joint which is affected and do a PEMS examination. By examining while the child is presenting we try to decipher whether we are dealing with something which is more actual joint involvement which is more like a larger root joint involvement.
Like example if there is a hip joint involvement or a shoulder joint involvement or knee joint involvement, ankle joint which are large joint or we are looking at the peripheral small joint involvement of the uh proximal interfallenial joint or distal interfallenial joint. So this helps us to narrow our differential diagnosis. Do not miss a good general examination which is very important when we are seeing ask the examine the uh eyes like already kupcha nanisar has mentioned that I oftal examination is very very important when you're dealing with a child with chronic arthritis a good skin examination mucosal examination a patch of alopecia if there is then your differential diagnosis may change and a good systemic examination whether there is any lympadinopathy associated with fever and joint swelling if there is hepatosplenomegalia associated or not these very important things when we are examining a child with a joint swelling in our OPD. So coming to a few cases here. So we did a simple this child came with a symptom suggesting of pain in the left wrist joint. We can see there is a swelling in the left wrist joint which is very evidently uh visible. But when we did a full PAL examination, we see that the right shoulder joint movement is also affected. But it was not the primary troubling joint for the child.
But just a simple PAL's examination helps us identify that the right shoulder joint is also uh affected in this child. Coming to the general examination. So if you it is very important to examine the nails because simple seriatic arthritis parents may never complain. They may think that nails problem. So they never complain.
This is very significant. But simple nail pitting is there or if you undress lot of times seriatic skin lesions are just miss covered up like a fungal leion. So parents always see a fungal infection here but they may be seriatic skin lesions. So it is very important to examine top to uh tip to toe and undress an examination examine ask for history of any skin findings or skin disease which the child might be suffering from previous to the joints uh joint symptoms as well. So this is very important. Once we have done all these good examination and a history taking we should be able to answer four things whether we are dealing with a articular or a periular swelling whether we are dealing with inflammatory or non-inflammatory cause acute or chronic and which kind of joint are involvement involved because for any joint swelling it is very important to recognize the pattern. Coming to articular perioticular like the first figure which I showed usually articular pain is more diffuse and a deep pain compared to perioticular which is more focal and is present only in few planes whereas articular is present in all planes of joint movement.
If it is acute always remember three very important cause which usually presents within first few days to two weeks of history which is the septic arthritis or osteomiitis with septic arthritis history of trauma of course and coagulopathies like I mentioned and in chronic cases of course infections and then juvenile idopathic arthritis is one of the commonest cause of chronic arthritis in pediatric age group.
Inflammation but simple symptoms of pain in the morning. Whether there is swelling or not, whether it is you're dealing with a more of a bony swelling or in fluid inside the joint. Signs of inflammation should be noted in systemic features which help us to identify whether it's a non-inflammatory pain or an inflammatory pain. Non-inflammatory pain can be from disysplas which may be non-inflammatory causes of joint pain.
So a good history and examination gets your half job done. We always see whether it's a well child who has trauma or no trauma. So this helps us in differential diagnosis. Sick child who has fever or no fever. So sometimes without fever there can be certain autoimmune diseases where very very mild fever is there and can present with joint swelling. So that can be a differential diagnosis. So always see whether the look of the child which helps us to identify the differential diagnosis. So if we are dealing with a chronic arthritis a juvenile idiopathic arthritis then the pattern of the joint involvement will always help us to identify whether we are dealing with a oligo poly if we are dealing with fever it's a systemic ja what kind of arthritis we are dealing with after we have a differential diagnosis in our mind it is only then that uh that we try to investigate and certain investigations always help us to rule out whether we are dealing with inflammatory or non-inflammatory joint pain. So for diagnosis like already mentioned there is no s no one test that will help us to identify that this is arthritis. It is a clinical picture and the history and the examination which leads us to diagnose arthritis. These are always supportive test casetoase basis test that we should do. So already mentioned ANA should be there should be a pre-est probability but most of the arthritis cases especially like sir had mentioned kupchaani sir that oligo articular ji we should always to do aa test only if we are dealing with polyarticular gi then we tend to do a rf test no blanket panel investigation should be done because most of the panel investigations are made for adult population rather than a pediatric population and of course sometimes tonography comes to our help to identify and uh the arthritis in the children. So in this we see ANA holds a very important position. If we are dealing with an actual joint involvement, we may tend to do a HLAB B27 test which is a sinister of juvenile ankyloining spondilitis. And one thing which I would like to mention as a poll here is RF test rheumatoid factor or anti-CCCP test is always done when we are dealing with something which is a polyarticular disease. Okay. So not all jis are RF positive or not all jis rf positive rather it is only 5 to 8% of jis which are rf positive also to mention not all rf positive may be ji a clinical picture is very important and sometimes in rheumatology diseases evolve over time so coming to case there is a this is a 7-year-old child pain and swelling of the right ankle for 7 days intermittent fever which was not documented but the parents were giving paras more four times a day to relieve the pain and fe uh fever which the child was having. So we see here it is an acute history, a sick child, fever and a single joint involvement. So we know we are dealing with something which is a rheumatological emergency here and we remember these three things which we initially mentioned a septic arthritis.
There was no history of trauma. So we are not considering trauma as the cause.
It was a female child and here we have bacterial infection as one of the most important things. So in this child you can see the whole tibia is involved and even the growth plate with ankle joint.
So there is a reactive arthritis septic arthritis with osteomiitis which the child is this is an emergency needing drainage and immediate IV antibiotics here. So simple history will help us to know that what we are dealing with three-year-old child coming to a second case a three-year-old child who had pain in both the hands for last two months.
And now we all know parents will first go to Google and ask. So they did Google the first test that should be done for joint pain. They did an anti-CCCP and RF which was negative. So they felt the child doesn't have arthritis and they kept on kept on taking some uh painkiller medications and finally they landed in the clinic. So this is the child where we can see the right wrist is completely swollen. She had a right wrist arthritis. Not only that by that time she developed a left knee arthritis. So we had dealing with toddler child who had a chronic disease for more than 2 months with two joint involvement. So we were dealing with a chronic oligo articular jia on investigation she had a high C reactive protein. Again reiterating the fact she had a positive ANA and when oftal examination was done she had a chronic anterior uvitis. So less than 7 yearear-old child ANA positive uvitis screen is very very important. And these child thus this child was put on anti-inflammatory and imunosuppressive medications.
Similarly a six-year-old child with pain in bilateral hands for 2 years. So we are dealing again with the chronic but it was bilateral hands and multiple joints were involved. A systemic examination was normal. So we were dealing with a chronic polyarticular disease. We can see the picture of this child where we see the proximal interfallenial joint. The wrist and the elbow all the joints were involved. So we were dealing with a polyarticular juvenile idiopathic arthritis in this child. So what is the map? Finally the road map that we go first we need to answer ourselves that whether we are dealing with a articular or perioticular. If it is a perioticular cause then we deal with a that kind of differential diagnosis. If we are dealing with articular we see whether we are dealing with something which is acute or a chronic disease and see whether it is inflammatory non-inflammatory and what pattern of joint we are seeing. If it is a chronic disease then we see of course the signs of inflammation is present or not. So if it's non non-inflammatory arthropathy if it is inflammatory arthropathy depending on the number of joints or depending on the pattern of joint involvement we classify the child as to what we are which kind of juvenile idopathic arthritis we are dealing with just a few slides on treatment. So this is a 13 year 3 months old female child pain in bilateral ankle and knee and right wrist for just one and a half months with fever had a low hemoglobin and an investigation was done which showed RF positivity and then because of RF positivity the child was referred but here it is very important to revisit the history when the when we revisited the history and we revisited the investigations we saw that the T and a repeat investigation showed a fall of TLC it was lymphopomin platelets were falling and a uric acid was very high.
So I'm reiterating the fact that sometimes RF positivity you have to have a clinical picture. This child had lymphodenopathy, hpatosphenomi, night symptoms very important. Leukemia can present with joint swelling. So it's important to examine and uh investigate in that line also if you have a differential diagnosis is m in mind because if steroid are given in this child it will not be good. So too much of a good thing is always bad and we should always know when not to use steroid. So not all jis need steroid. In fact jis do not need steroid. It needs more of a disease modifying antiroatic drugs IGA vasculitis. So for that sake empirically steroid should never be started and in the long term if you are starting steroid you should know that steroid sparing molecule has to be started along with it. This is a very important thing we should remember when we are dealing with a child with arthritis. a wholesome treatment with dear whenever required biologics and never forget the uvitis screen even if the child is not presenting with any symptom because it's a chronic anterior asymptomatic uvitis with the child may have a wholesome treatment with oftal evaluation a calcium vitamin D supplementation adequate physiootherapy and exercises and explaining the parents about drug side effects because it's the primary pediatrician it is the rapo building where the child or parents have and they will always come back to the pediatrician to ask whether they are taking the drugs correct correctly or not. So in a nutshell we should not treat in a haste. Do not send tests which are misplaced. Examine thoroughly but hear the history primarily. No steroids, no panel investigations, no steroids empirically, no panel investigations, pattern recognition and then evaluation. That's most important because what we use the carpal bones to identify the age the arthritis can cause that damage to that child. It's very important to understand if not treated in time there is a huge amount of damage that can happen to the joint of the child and primary care provider pediatrician will help in diagnosis vaccination intercurrent illness counseling of the family drug monitoring all has to be done by the primary pediatrician. Thank you.
>> Thank you madam. I think all pediatricians should be confident now to have approach to joint swinging in OPD.
Uh Kuchchani sir your expert talk sir please unmute sir please unmute.
So Shika that was spot on as usual.
Uh first I must reemphasize some of Shika's uh messages. Identify emergencies like a septic osteomiitis or a septic arthritis.
She also mentioned about trauma. But I must say one thing to you that every patient who comes to the rheumatologist will say school bench start.
So do understand the time duration between the trauma and the onset of symptoms. And invariably if it is trauma the latent period should be in minutes to hours not after days.
The third point is about pain. We have to recognize that pain expression is a milestone that comes only after approximately 4 years of age.
So children below three and a half or four may not express pain but may indirectly show pain by either excessive crying, irritability, limping and such maneuver. So you got to be cautious about that. Again, Sika, thank you very much for reminding the audience about no steroids.
It is still very unfortunate that the point of first hold for any patient with a limb pain is a orthopedic surgeon. And this is where as pediatric rheumatologists not only have we to sell our services but we have to also sell our branch and this becomes rather important.
She again reemphasized don't do rheumatoid factor CCP HLAB 27 as a screening process. And I want to end with just two points. One that when we say oligoarticular involvement, it is different from olgoarticular ji.
So if I'm talking to Sika and saying I'm seeing a child with oliggo articular involvement what I mean is less than four joints are involved but when I add the adjective or the noun ji then oligo articular becomes an adjective to describe the type of ji that I am dealing with. So, and again to reiterate that the causes of arthritis are in a memonic called arthritis which you can easily found find if you do a Google search. It's the end result of things from infective endocarditis right up to inflammatory bowel disease.
And therefore what she said about history taking right from when the child enters to gauge the age and sex of the child is very very crucial. And I will end with one case that just was a masterpiece after which we have seen four such patients.
This was a warm joint but completely painless.
And this was troubling us. Long story short, this child had a hereditary sensory neuropathy with a shakos joint.
And last but not the least, if you see a child with autism and a painful joint, just do an X-ray to look for scurvy.
>> Thank you.
>> Thank you, sir. Uh Dr. Sanju Jooshi has joined present, sir.
>> Yes.
>> Yes, sir.
>> So extremely sorry. Extremely sorry for joining late. Am I audible?
>> Yes sir. Yes sir.
>> Yeah.
>> You and I express my sincere gratitude to uh nobody else but the teacher of teacher the clinician par excellence and uh country he's he is none other than Dr. Rajupandani.
I know one of the tagline for uh the iPhone when it it was launched uh in the earlier days that uh uh they used to say if you don't have iPhone then you don't have iPhone or I would say that if at all we are discussing pediatric rheumatology and if you don't have Rajuk Chandani then then you don't have Rajuk Chandani and I also uh acknowledge the presence of my mentor and my inspiration always from the res residency till date uh till today is uh none other than Dr. Girish Subramanyam. Thank you Girish for uh for having a great impact on my academic journey over the years and of course I just listened to Shikha's deliberation.
Excellent Shikha uh she's not only the dynamic and most versatile secretary of Mumbai IIP but she is also a astute clinician. I also welcome the another younger Dr. Himmanshi from Pune and of course I am pretty sure that uh today's session will definitely empower our practicing pediatrician across the state. uh it will help to in uh improve their clinical acumen uh in the field of pediatric rheumatology. how to optimize the lab use and when to refer uh uh as always and al also uh the important message Shika has given as Raju has already elaborated is never ever use bland blindly steroids in uh all the arthritis. This is a very big message Shika. So I uh I don't want to interrupt between the science and you all. So let us dive again into the ocean of knowledge. So over to you Kundan. So again thank you Raju sir uh Girish for accepting our invite.
>> Thank you.
>> Thank you. Thank you our privilege >> sir. More than 100 delegate pediatricians have joined.
>> Yes. Yes. without MM the name itself is explains everything here and I also acknowledge the presence of the uh dignitaries and the luminaries from the CIP like Dr. Jalil sir Dr. Savar Agraal sir Dr. Yogesh Parak and so many of them are there. So welcome all. Thank you all. Thank you. Yes. Signing off.
>> Thank you.
>> Yes. We will move to our third talk that is from Dr. Himman Shu. Himmani Chowri that is on Kawasitki disease typical atypical and complete incomplete and atypical. Over to you ma'am.
Slides are visible.
Okay. So, um so good evening everyone and uh thank you MahaP for this wonderful opportunity especially um to talk about a topic which is very close to my heart which is Kavasaki disease. a topic um which is as uh relevant to a rheatologist as it is for a pediatrician. Rather um my mentor who I will be talking about later always says Kawasaki disease should only be managed by a pediatrician. So thank you very much for an opportunity and um moving on. Uh so the first u description of Kawasaki disease came way back in 1950.
So the disease is named after the famous pediatrician from Japan Dr. Kavasaki who reported the first few patients of Kavasaki disease in 1967.
It was such a novel disease he could not name it. So what other people named it was mucaneous lymph node syndrome.
I'm very proud to acknowledge the fact that the second description of Kawasaki disease in India came from my city Pune itself which was in September 1988. This was um when Dr. Nitsur reported a child who had multiple arterial aneurysms and was diagnosed with a classical Kawasaki disease. So this was way back in 1988.
So coming to um a very very important question how common is Kawasaki disease?
U I have seen patients I've seen people I've seen pediatricians referring it to as a very very rare very very uncommon disease. So just the facts here that how common is Kawasaki disease? This is an old paper. Um so the data here is for about 2012 >> and as you can see here um the highest incidence is actually highest incident is reported in Japan and other Asian countries uh where the incences >> ma'am I think your slides are not slides are not >> can you can you reshare the slides ma'am >> yeah that's >> so can you see the slides now >> no >> okay I'm going to just reshare I think you have to stop sharing and reshare.
>> Yeah. Yeah, you have to reshare.
>> Okay.
>> Yeah. Screen is visible. Yeah. Yeah.
>> You can make it full screen.
>> Full screen.
>> Yeah, it is seen.
>> Okay. So, how common is Kawasaki disease? So the communist um pre the commonest incidence of Kawasaki disease comes from Japan and the nearby Asian countries where it's reported to be as common as 264 in 100,000 children. So if you simplify it uh Japan will see Kawasaki disease in around 1 in 500 children less than 5 years of age. And when you see in India the uh incidence that was reported way back in 2012 that was 4.5 in 100,000 which is around 1 in 20,000 children and there is a recent uh paper where there there have been projections that how common uh I mean what would be the uh incidence of Kawasaki disease by 2030 and as you can see uh in this paper they have projected that Kavasaki disease will see a monthly increase of 0002 cases. per 100,000.
Mind you, this is a monthly increase. In children age less than 5 years and in children if if you calculate in children less than 15 years, the incidence would be as high as 0165 cases. So this is a monthly increase per 100,000 children.
So how common is Kavasaki disease? So KD is now the commonest cause of acquired heart disease in children in many countries and it is the most common vasculitis.
So uh we we are all aware of the diagnostic criteria wherein a fever has to be present for at least 5 days and when you see four out of the five conditions which is conjunctal injection a polymorphus rash cervical lympadinopathy mucosal changes and some limb changes. So in the acute stage you can see edema edema of hand and feets and in the convolescent stage once the fever has passed away you see perangual desquamation also a point which is very important is an illness which is not explained by any other cause. So this is the diagnostic criteria which has been given by American heart association.
So this is a slide which you know I have made by myself u as per uh my education as per my experience with Kawasaki disease. So diagnosis of KD is more art than science. Diagnosis rest on the recognition of a temporal sequence of characteristics or clinical findings.
These clinical findings may change from day to day and there is no pathog test that can tell you whether the disease is Kawasaki disease or not. So what is important is a careful history which may reveal that more than one principal clinical features were present during the illness at any point of time but they may have resolved by the time of presentation. So it's very important that history is the key in making the diagnosis of Kawasaki disease. So let's talk about something which is typical which is not so simple but yet typical Kawasaki disease. So coming to the first criteria which is fever for 5 days. So what kind of fever do we see in Kawasaki disease? Usually a high-sp fever which is remittant keeps on coming back and forth. First day of fever is considered first day of illness. Duration of fever is generally 1 to two weeks in the absence of treatment but it can even go on for 3 to 4 weeks. With treatment fever generally resolves within 1 to two days after therapy.
Next feature which is conjectal injection. It usually appears with fever. It's more bulbar than palpibbral.
There's a characteristic perilmal sparing and there are no exudates. It may last for one to two weeks and sometimes you can even see a uvitis which can present with the conjuctivitis. Subconal hemorrhage and punctate keratitis could also be seen.
Then there is a host of oral or fngial changes that could be seen like lips could be red, dry, fissured, peeling, bleeding. a tongue the characteristic strawberry tongue could be there where you have prominent papa and arythema and you can also see fngel iryma uh it's important that you don't see oral ulcers which oral ulcers are not characteristic of KD so is fngel exudates or copic spot so these are not something which make which will make you think of kawasaki disease then coming to lymph nodes so cervical adinopathy it's usually anterior cervical lymph nodes node may be more than 1.5 cm in diameter making them pretty significant ificant may or may not be associated with overlying arythema. The nodes are usually not fluctuant or purulent and they may be unresponsive to antibiotics.
Now rash rash can take many forms. It could be a diffused maculopapular arymatus rash. Sometimes it's very extensive primarily involves the trunks and extremities. It could be urticarial or you can even see fine micropostular eruptions. Scarletiniform rash and an arythema multifiform like rash with target lesions can also be seen. So practically any kind of rash you can see in kavasaki disease except a vesicular rash which is not KD u then you know you can see a diaper rash you can see arythema and desquamation in the groin area which is frequently mistaken to be a candidal diaper dermatitis. So again it's very important when you when we see very small babies when we see infants with high spiking fever um which is unexplained you actually strip them down see the diaper area and and look for a rash which could be characteristic for Kawasaki disease. Peranal desqammation again this is a very very classical peranal desquamation of Kavasaki disease usually seen in acute phase of illness.
I mean rash this desqueamation can be as bad as this and this can even be labeled as soriasis.
What is not what is not characteristic of KD a bullus a vicular and piticial rash is something that would not make me think of kawasaki disease. Then what are the findings in hand and feet and it is very distinctive. So arythema which is confined to the palms and soles and even sometimes parents t tell you that hands and feet may be marketkedly swollen as if some salign I mean as if the patient has received some infusion in that uh arm. Infant and children frequently refuse to hold objects in their hands or to bear weight on their feet. In subaccute stage of illness you will see perangual disquamation of fingers and toes and about one to two month later you can see something which is called as a Bose line which are deep transverse grooves across the nail. So these are typical findings of a patient with classical Kavasaki disease.
Okay. So we've discussed what was classical about Kawasaki disease. Why should Kavasaki disease be treated?
Because missing a diagnosis of KD can have disastrous consequences. It can leave behind severe cardiac squ. So what are the cardiovascular complications that we are worried about? So it can cause everything starting from myocarditis, starting from cardiac shock, coronary artery abnormalities which are characteristic. So coronary artery abnormalities can present as coronary artery aneurysms and they can even present with coronary artery thrombosis. So what happens is 25% of untreated patients with Kawasaki disease can develop these cardiovascular complications but as per studies only less than 5% of children who have received treatment on time would develop these complication. Hence it's imperative that you treat Kavasaki disease before the coronary complications develop. So it's very important to treat Kavasaki disease before those 10 days of fever. that is when you can actually prevent these complications.
So what usually so what is the course of Kawasaki disease? So um a child will will present with fever. He will have a range of other clinical manifestations like oralom mucosal changes, some limb edema, some limb changes, some rash, some lymph fedinopathy and that is when he can present with myocarditis and this is the phase when you will have high inflammatory parameters but the platelets will still be normal. After 10 days that is when the cardiac complications start appearing. So as so as platelets start rising uh that is when the risk of coronary abnormalities also start increasing. So um you should not target to treat Kawasaki disease only when coronary complications have developed. So sometimes I get a referral from people who tell me that you know this this patient with uh you know with with some prolonged fever with some clinical manifestation with high inflammatory parameters looks like Kawasaki disease but we've done an echo and echo is normal. So this does not look like Kawasaki disease which is very very wrong. So Kawas I I keep telling them that uh you know you you you should I mean ideally you should be treating Kawasaki disease before the coronary complications develop. So this is the message. I mean I'm going to repeat it in the end but this is the message that I want to pass that um I mean you should treat Kawasaki disease even if they have complications but our main aim as pediatricians as rheumatologists should be to treat Kawasaki disease before the coronary complications swell up.
So we are well aware of the differential diagnosis the the clinical spectrum of conditions that can present with fever with rash with a lymph node. So you can have viral infections like adino virus, measles, herpes, um leptospiral illnesses, strep, stafylocus, especially toxic shock syndrome can present with a picture very similar to Kawasaki disease. Hypers sensitivity reactions like SJS, Steven Johnson syndrome is can one can be one of the differentials.
Also drug hyper sensitivity syndromes can present with fever, rash and then there's a host of other rheumatological condition especially systemic jia which also comes in young children can come with a rash can come with lymph nodes can come with high inflammatory parameters and can mimic the Kawasaki disease. So what happens in investigations uh when you see the CBC you'll see uh elevated TLC and you will see uh platelets which start rising after uh week two of fever. uh there will be elevation of acute phase reactants. So ESR and CRP are elevated.
You will see hypoalmia u and you know of course we do an echo which can show us uh myocarditis which can show us coronary abnormalities which can show us pericarditis. So all these things are very very important also.
What is important is you just don't echo in you just don't say that the echo is normal uh in the initial evaluations. It is also important to repeat it periodically if you've made a diagnosis of Kawasaki disease. So an unavailability or technical limitations of echo should not delay treatment of KD. There are centers there are there are places where a cardiologist is not available uh for a patient who's admitted in the night or you know he he's just not there. So um the the fact that you cannot do an echo should not be a limitation for you to treat KD. So a di so a cardiologist should not be uh should not be like the most important factor for you to take a decision on treatment of Kavasaki disease. This disease has to be treated by pediatricians.
So how do you treat KD? The goals of treatment are reduce inflammation and prevent thrombosis. So how do we treat it? We treat it with IVIG. So 2 g per kg single dose single infusion. Again I've seen uh places where um where the dose of IVIG is given over multiple days but as per uh the guidelines as per um the regime it is 2 g per kg as a single infusion. Aspirin uh we have moved away uh from the practice of giving high anti-inflammatory dose of aspirin. So now we use uh the anti-thrombotic dose of aspirin which is 3 to 5 mg per kg per day. So again I'm repeating the very same thing. Um and this is something which is repeated again and again in the AHA which is American Heart Academy paper on Kavasaki disease. So it is very very important that you diagnose KD within 10 days of fever onset. Ideally if you see a patient with complete KD you should diagnose him within four to five days of illness and at least within 10 days if you see a child with incomplete KD because it is the treatment within the 10 days of fever onset which will uh eventually decide whether the patient would have coronary complications or not. Okay. So now u there is emerging data that in some cases IVIG may not just be enough and you might need to intensify therapies.
So what are the high-risk groups wherein we intensify the therapy? I mean by intensification I mean beyond IVIG you add something else as well. So in infants especially the very young ones the less than 6 months is a very high risk group wherein you see that where there's a high incidence of coronary complication high incidence of incomplete KD. So these are the patients who are given additional therapies besides IVIG. The ones who have coronary aneurysms in the first echoes. Again those are the ones who have to give a higher uh imunomodulation and those who present with severe complications like KD shock syndrome or macrofase activation syndrome. So this is the subgroup this is the high-risisk group among the patient wherein probably IVIG is not enough and you need to intensify therapy with with other immodulators.
And what are the imunom modulators that we use are our friendly steroids. Um then we also use TNF inhibitors like infleximab it we also use IL1 blockers like anakindra and in some patients we also use cycllosporin.
Okay. So um I think we have talked about uh something which we all know. So now I'll be touching upon something which gets missed uh which goes undetected which which lands up in um a lot of complication which is incomplete or atypical Kawasaki disease and this is um this is that area where where you know there's a lot of discussion there's a lot of argument uh especially when when when you're dealing with a sick child and that is when um you know taking quick quick actions is very important.
So um when do you call something as incomplete Kavasaki disease? uh so you have unexplained fever for 5 days but the entire diagnostic criteria for KD is not met. So those are the ones where you label them as incomplete Kawasaki disease. So again I am again going to reemphasize that treatment and management of Kawasaki disease is under the purview of a pediatrician. So all pediatricians must be familiar with the extended spectrum of Kawasaki disease.
So incomplete KD it is common in infants. the risk of coronary abnormality is comparable rather higher than classical KD because it goes missed and you have to pay attention to weak findings. So this is uh the uh algorithm which which we follow. So children with fever for more than 5 days and two or three compatible clinical criteria.
Another very very important thing is if you see an infant with fever for more than 7 days without any other explanation you do inflammatory parameters and you see CRP is high ESR is high of course you've ruled out other things and then you see the other things like there could be anemia thrombocytosis which has started coming after 7th day of fever you see low albamin you see some transmitis you see sterile pyura or you see an echo which shows coronary abnormalities treat those patients I mean IV VIG these days is very very accessible everyone I'm sure you know almost everyone have access to IVIG uh in this date or time and IVIG is not as unfriendly as steroids it's not something you know which which might cause very something really bad to a patient so I think it's something which should not be denied to a patient where you're considering a possibility of Kawasaki disease so what are the some other clinical features of KD that you should be aware of may often so some things which could come with KD you can have myocarditis of course you can have arthritis of course thrombocytosis starts appearing in the second week and things which are pathogic of Kawasaki disease so the first one is reactivation of BCG scar mind you it's pretty common so in my in my in my own practice I have seen so many especially infants some of them even come with a presenting feature that you know uh there was some redness in the BCG site and then the fever started coming. So it's something which is very very pathogamic of KD and should not be ignored. Peranal descation again very very pathogenomic of KD. Sometimes we do an ultrasound where children have vague complaints and you saw when you see a GB which is distended a gallbladder hydrops is very pathogenic of KD and sterile pyura again very very pathog.
So peranal peeling it appears earlier than perangual peeling and reactivation of BCG is almost pathog. So KD is a vasculitis. Vasculitis systemic vasculitis all vessels of any practically any organ of the body can get involved can get inflamed and can cause symptoms. So you can have symptoms which could start from the brain could could end up with the heart, the lungs, the liver, the kidney, the muscles, anything can practically get involved in a patient with Kawasaki disease. So this is a very very um classical Kawasaki disease which I saw very recently. So this was an eight-month old girl. So she was uh the mother was basically a doctor and um she saw a BCG site reactivation uh in the baby. That was the first presentation. The child also continued to have fever despite being on antibiotics for some day. Uh the mother characteristic said that that she developed some conjectal congestion on day one but it resolved because she was so observant. She also said that there was a faint macular rash which I noticed around day five, day seven of fever and it resolved. It's it was no longer there by the time we presented in the hospital. She did have some red chapped lips, oral edythema, but the doctor said she she was not feeding properly. So probably she has some dehydration and she'd been treated with antibiotics from day three onwards. So they uh did her 2D echo on day seven and it was normal coronaries. So the mother asked the pediatrician could this be Kawasaki disease and the pediatrician admitted her did all these tests. test showed um some anemia, luccoytosis which was polymorphic, uh platelet counts which were okayish on day seven, ESR was high, CRP was high. So they kept the patient um you know give gave some IV antibiotics, repeated test on day 12, she was a fibral for 24 hours and uh the CRP had fallen to 18. So she was discharged on day 13 because the pediatrician said that the CRP is falling. There is no coronary abnormalities. This does not look like Kavasaki disease. Go home. It's an infection which has been cured by antibiotics.
And this was this was a doctor mother.
So she came to me on day 14 because the child again developed fever. So we we we readmitted her and um day 15 investigation showed a TLC which started rising, platelets which were further up, ESR which was elevated, CRP which was still up. We did an echo. Thankfully she did not have any coronary abnormalities.
Should we or should we not treat her? So um I will answer it for you. So all Kawasaki disease patients need to be treated unless and you know regardless of whether they have coronary abnormalities or not. Only 25% of Kawasaki disease patients develop coronary abnormality. Doesn't mean you don't treat the rest 75%. So if you're making a diagnosis of Kawasaki disease, you treat the patient because the risk of coronary abnormalities goes beyond deference of fever. So you can have coronary abnormalities till 6 to 8 weeks of fever. So it's very very important that you treat all children with Kawasaki disease and and this is what I saw uh when she came for follow-up. So this was a very very classical very classical perangle peeling that I saw but again this patient had no coronary abnormalities. This is an 8-year-old boy who presented with fever for 5 days, had a maculopular rash for 3 days, excessive irritability, altered sensorium, hypotensive shock, uh he had dorsal edema over bilateral hands and feet and a big liver. So this was basically a patient who who was labeled as myocarditis and um we we treated him as Kawasaki disease because of the other features that he had. He had a very very characteristic conjunctal congestion. I mean if you see here this characteristic peri limal sparing and you know eventually he also peeled kind of uh nail in the coffin that this was Kawasaki disease. So when we when we did investigations as you can see initially in the first week there was actually thrombocytoenia. So I mean if you see this patient he had some anemia he had low counts TLC was low platelets were low ESR was high CP was high we done a blood culture and echo showed uh a zed score which was I mean it was a mild coronary aneurysm with a mild pericardial eusion but the ejection fraction was just 38%. So this patient had Kawasaki disease shock syndrome. He had frank myocarditis which is very common uh which is common in patients who present within the first week. So um this is again a statement which is uh given by kavas by by AHA which which says that presence of thrombocytoenia and coagulopathy in a child who presents with myocarditis presents with acute history it should be noted and when you have ruled out a possibility of bacterial sepsis then you should consider a possibility of kawasaki disease in these patients and these patients should be treated. So these are one of those cases where we we get into a tiff with um ICU consultants but this was a very very um well-managed patients and of course I mean um a lot of credit to the ICU consultants for managing such sick patients. Um so this was a three-year-old boy who had fever for 20 days, maculopapular rash, edema over both hands and feet, cracking of lips, redness of eyes. So this spoke called Kawasaki dis disease. What did not speak of Kawasaki disease was that there was yellowish discoloration. There was jaundice. There was darkening of you. There was frank jaundice. He had hippatospromegali. He had a sitis. He he practically had hippatic enkapilopathy.
Uh we did an echo. It showed an aneurysm. So this is a patient who had acute hepatitis. He had acute liver failure because of Kawasaki disease.
Improved with treatment. This is another classical case. a six-year-old boy who would present with a short history of fever would have been treated by multiple antibiotics with a uh will will develop a sudden uh you know big cervical lymph node u and an FNA was also done for it and it was reported as a reactive hyperplasia this could again be a presentation of Kawasaki disease this need not always be bacterial edinitis this could be Kawasaki disease okay this is another girl so again admitted in the ICU six-year-old girl high-grade fever for one week had some chapped lips. Mother said had a macular generalized rash. She was admitted with neck stiffness. She was not moving her limbs and she was very irritable. Uh so they considered a possibility of menitis. Did a CSF uh which show 10 cells which were lymphocitic. The entire infective workup including a CSF biohire was negative. U they also noted that she also had polyarthritis and she continued to be treated with antibiotics.
Um when we saw the investigation there was some anemia luccoytosis platelets which had started rising a high ESR high CRP and CRP look at the CRPS here they're like in the range of 150 to 180.
We did a 2D echo on day 12 and it showed giant aneurysms in all the coronary arteries. So this was one u atypical Kavasaki disease which was missed because she presented um with neck stiffness. So what she actually had was polyarthritis. So even the neck stiffness was a part of polyarthritis.
So um you know not all even patients with Kawasaki disease can present with arthritis can present with a sterile aseptic menitis.
So this is another kid 18-month old girl fever for 3 weeks excessively irritable urine examination was done which showed pyura and she was treated for UTI. She had a generalized rash which was labeled as drug rash and all investigations for a bad UTI were done including an MCU. uh when we saw the investigations again she had high ESR high CRP luccoytosis had characteristic peeling again especially in infants sterile pyura when you see an infant with fever with you know when you've ruled out everything else just bad fever which continues despite giving antibiotics this need not always be UTI this could actually be Kawasaki disease and she also had aneurysms so this is a case from um PGI so Uh this is interesting because this is um an adult Kawasaki disease. So this was a medical student who was a 30-year-old girl with fever, characteristic red eyes, characteristic tongue and she had a generalized body rash. She also had a lower motor neuron type facial paly referred from the adult rumat because they were aware of Kawasaki disease and and this is what it it looked like. Uh thankfully she did not have any coronary abnormality. She was treated with IVIG because uh she was an adult. We considered her as high-risisk gave also in fliximap and and she was very well after the treatment. Talking about a 9-year-old girl um she had so this is again a patient of mine. So she had fever for 15 days. She had red eyes. She had phototohobia and she had a rash after fever and both eyes showed uvitis.
So again this is acute uh uvitis presenting with fever. When you see the counts it made me think of Kawasaki disease and even Kawasaki disease can present with uvitis. um another patient of mine who had uh basically an acute onset hearing loss with Kawasaki disease. So when we talk about Kawasaki disease, it's a vasculitis and again I'm just reiterating it can involve any organ, any part of the body.
Uh this is another patient with myocarditis, coronary artery aneurysms.
So um if you could just look at the table you you'll see like you know he had falling counts he had falling platelets transmitis um high TGS low fibonogen a a picture which could make you think of HLH and bad fevers a fat fever which is just not going away. So this was a child with Kawasaki disease who developed a complication which is called as macrofase activation syndrome. So this is Kawasaki disease which can complicate and can also cause the severest life-threatening complications for us which is macrofase activation syndrome and and we've seen children um with macrofase activation dying within 24 hours. So this is again something uh the inflammation can go completely out of your hand and can cause lethal and and really bad complications. So every child with KD uh can worsen.
So just just I'm going to summarize what are the common pitfalls in diagnosis of KD. If you see an infant or especially if you see a child less than 6 months of age who has prolonged fever which is unexplained is irritable it could be the only clinical manifestation of KD and these are the ones these are the ones who are at the highest risk of developing coronary artery abnormalities. U children more than 5 years of age and adolescent again this is a high-risisk uh group because you never think of KD in older children. I mean I mean we think of KD as something that comes for younger children but again uh KD can present in older children and uh they have a delayed diagnosis because you know we are programmed that KD is for younger ones.
So again they have high prevalence of coronary artery abnormalities. If you see an infant with prolonged fever sterile pyura who has been treated as UTI and there is no response to antibiotics it could be missed KD. There could be a possibility of delayed diagnosis and high risk of coronary complications. fever, rash, red eyes, red lips.
Uh sometimes these kids are treated as drug rash and again this this could actually be missed KD. Prolonged fever, cervical lymphadinopathy, something which is usually treated as bacterial adinitis but you see no response to antibiotics, think of Kawasaki disease. A rash which comes with hypotension, fever, rash, hypotension which is treated as toxic shock syndrome but is not improving.
Think of Kawasaki disease. An infant prolonged fever, irritability, culture, negative CSF, pioytosis which is who is treated as menitis and no response to antibiotics. Again think of Kawasaki disease. An infant rash GI symptoms sent to surgical services could be Kawasaki disease. So when should you suspect kavas disease in children? So any child any child with recent onset of unexplained fever a fever which is just not going away despite all tests being negative think of KD and any infant. So these are the ones where we have to be extra precautious. Any infant who continues to have high-grade fever we have to be very very conscious about Kawasaki. should be Kawasaki disease should be in the differential diagnosis of these children. So take-home message um I hope I'm able to convince you that it's a common childhood illness. Beware of young children with unexplained fever for more than 5 days. KD can often be a diagnostic nightmare and uh I mean um let me tell you a few of my patients are actually awaiting a bypass. They are just 12 and 13. uh they actually planned for a cardiac bypass because of this complication. KD is rather unforgiving.
Missing out on a diagnosis can have disastrous complications. As I said, uh some of my uh patients, I mean, we're actually lining them up for a bypass at the age of 12 or 13.
So, uh this is my mentor, uh Professor Singh, um who who in the other picture you can see him with Dr. Kawasaki himself and uh um it's it's a great honor it's a great privilege to be uh speaking u you know on this topic which which has been his topic u throughout his clinical practice and um this is my clinic uh I I do only uh immunology and pediatric rheumatology um at Pune so this is this is my clinic thank you so much thank you for joining us thank you thank you ma'am thank you Dr. Chandan sir, your expert coming please.
>> Yeah.
So a talk on Kawasaki could have only been given by somebody who is a PGI alumnus. And I must state that the very first case of Kawasaki I identified sometime in the year 2001 or two was only after I heard a lecture by Sjit in the Pata IP conference.
Uh lots of scenarios have been shown by Himmanshi. I would just like to add a few comments.
She has been very exhaustive in telling you every possible scenario in which you should suspect Kawasaki.
Suspect for sure, but please don't be in a rush to treat.
Identifying Kawasaki is an urgency but not an emergency.
with lectures such as this.
Pardon me if I am sounding overconservative but I am seeing a bit of overdiagnosis of Kawasaki which is reflected in the very massive increase in the sale of IVIG if you ask any of the pharma companies.
So it's certainly important and very often we get phone calls from periphery saying sir bar typical strawberry tong now that typical strawberry when I went to mahabeshwar two weeks ago the strawberries were quite pale over there so it then we are just using the word strawberry assuming that your strawberry and my strawberry are the same shade.
Okay. So my advice to all the clinicians is please start taking photographs at least on the parents cameras so that in case you need to reach out to Imani or anyone she agrees with your strawberry because now we have patients coming in saying sir he had a strawberry tongue and cracked fishered lips.
Now that they could even kind of generate from Google Geminac.
The third issue I want to state is that when you are sending the patient for an echo, please record the height and weight very very carefully and identify a proper pediatric cardiologist who is not too anxious to please you with a coronary dilotation.
He must tell you what is the Zed score and for that he will need the height and weight and I have seen pediatric cardiologists who record the weight on a bathroom scale that is not enough for you. You need absolutely because there is a formula in which they have to enter it and then there is a website called parameters where you will be able to get the Z score.
The other point that after you have given IVIG the ESR is not so relevant.
you need to monitor the CRP.
This is another important practice point.
Uh facial n policy I have reported a case in Indian pediatrics. Besides this hemorrhagic bilateral plural eusion is another atypical kawasaki. I have seen books have mentioned that one of the early signs to look for is to do an autoscopic examination and look for both the tempanic membranes. These are very vascular structures.
So look at those and the last point that is that we have to champion Kawasaki disease to our adult cardiology colleagues.
I was dumbfounded in my days in Jaslo about 10 years ago when I bumped into an adult cardiac surgeon and I told him that I'm managing a child with Kawasaki and anorisms. He said and it really stunned me and therefore uh one of my dearest friends, Professor Jane Burns from San Diego has created videos to educate adult cardiologists.
And about 6 months ago, I did a video consult with her for a 18year-old boy with giant aneurisms who was getting a green card to go to the US and I was asking her about rotabladder angoplasty etc. And what she said is Raju days have changed.
If your child had Kawasaki when he was three or four or five, we prefer to follow a kinder gentler approach because the collateral circulations develop so very well that these children with just proper lifestyle management monitoring their lipid levels and double anti-coagulation often escape any very heroic procedures.
Anybody who's interested in this video, please send me an email and I will forward it to you. But they are there on YouTube. Search for Jane Burns, University of San Diego and see those videos. There is one for the patients and one for the adult cardiologist. Very beautifully done 12 minute videos.
Thank you.
>> Thank you. Thank you sir.
So there are some questions. It is already 10:40. Uh so I will just read the questions for you. Uh Dr. Shikam ma'am there is one questions uh I think you have answered in comments also. What is >> I have maximally answered all questions in the comment. Please go ahead tell me now.
>> Yeah. What is PMS?
>> I have answered. So it's a regional examination of the muscularkeeletal system. So supposedly if we find the elbow joint is involved in pals, we do the exact examination of the elbow in all all planes of uh uh movements.
>> So that's the regional examination I've answered in the chat box.
>> Yeah. There is one question for him also. What is not Kawasaki but looks like it? I think she has given in detailed explanation also.
>> Yeah.
>> Which ma'am?
>> So um what I've mentioned wherein the fever would be there but you would not have the complete picture of Kawasaki disease. So you would not have you would not meet the criteria. So that is when you can have uh incomplete or atypical KD and and we've discussed how you know it can present with so many uh features.
So I I think we've discussed this already.
>> Yes. Yes. So it is already 10:40. So I will just like to thank uh today's expert Dr. Raju Kupuchchand Dani sir for his precious time and words of wisdom.
Then I also like to uh all speakers thanks all speakers Dr. Girisham sir Dr. Shikar ma'am Dr. Himi Chri ma'am I'd also like to thanks all office bearsers of my EBS of central IP and EBS of my also I'd like to thank scientific Dr. Ba ma'am and our scientific advisor Dr. Prashan Zadu and most importantly the our Pali ma'am for her technical support. I think we will conclude this meeting. President sir, secretary ma'am.
Thank you one and all. Thank you sir.
Hello sir always and Girish Shikai.
Excellent. Excellent sir.
>> Thank you. Thank you so much.
>> Thank you. Thank you everyone.
Dr. Kupchandani sir uh we have got a very good insight and you have done a very lot magic with your words for us.
Thank you so much for joining so late also. Thank you >> and thank you all the expert faculties today and also to our kundan and >> who have presented very well.
>> Thank you.
>> So we will wind it up with a good night namaskar sharatri.
>> Good night ma'am.
>> Good night. Good night.
>> Arma was feeling pity on me thinking that it's very late but I'm just getting ready to watch Durand 2. painful.
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