CASES OF THE WEEK – “Fever – with a race against time!” by Dr. Nisha Soares Consultant, Paediatrics, NMC Royal Hospital Sharjah
Fever – with a race against time!
A happy active 8 years old who has followed up with us from infancy, prone to allergies & infective URTIs came to the clinic with a low grade fever & itchy, scarlantiform body rash and the first thought was scarlet fever. He was not responding to presumptive treatment with antipyretic and antibiotics. Covid swabs and serology was negative but as the fever continued the child was hospitalized to monitor the fever - his CRP was unremarkable at 24 but his WBC was at 21,000 with platelets 456,000 & eosinophilia of 10%- the last being unusual.
His fever STILL continued, the rash kept spreading with some peeling & he complained of fleeting knee pains, his left eye was a little congested with a small possible strawberry tongue. ECHO was normal. His CRP increased serially 24 to 32 to 67 and counts as well to 28,000 – neutrophilic with prominent eosinphils 10% then 18% and platelets 560,000.The diagnosis was difficult as the child was very well looking – in fact happy, making multisystem autoimmune issues a difficult diagnosis to consider! There was a small possibility of viral infection but the blood picture made it less likely. His case was extensively discussed by the MDT but no denifite conclusion could be reached.
Finally, taking into account the multisystem nature of symptoms, the inflammatory markers on the blood picture ( though the CRP was not hugely elevated), a presumptive diagnosis of an atypical Kawasaki was reached (3 of 5 features with skin, eyes & oral mucosa). Since we were on the 7th day of the fever & with review of signs & symptoms, IV immunoglobulins were started.
The fever seemed to come down for one day, & then frustratingly was up again, making the whole diagnosis even more complex. The WBCs & CRP seemed to level o but the platelet count & eosinophils kept increasing. We were on day 10 of the fever and time was ticking by. With an extensive review of all the features & laboratory results, we decided to give a second dose of immunoglobulins on the presumption that the fever was inflammatory & kawasaki (though resistant) was still the most likely. The fever still persisted for 48 hours after that, though lower & then finally settled.
2 months later the child is ne & remains his happy self, serial ECHOs have been normal!
Since rst described by Tomisaku Kawasaki in 1967, the disease has been described in many countries & races outside of Japan. The etiology is unknown, though the consensus is an exaggerated response to an immune trigger.
It remains a rare disorder, with a prevalence of 25/100,000, 80% being under the age of 5. The problem with diagnosis, is that Kawasaki is a great mimicker of diseases – EBV, scarlet fever, systemic JRA being just a few named ones. There is no diagnostic blood test or imaging modality which leads us to the diagnosis except a practiced clinical eye! Even more dicult is the fact that treatment is best in the rst 7-10 days as Kawasaki can aect the coronary arteries & is the leading cause of acquired heart disease in children around the world – treatment on time is empirical. If Kawasaki is rare, ‘resistant kaswasaki’ is even more so. Dened as persistent or recrudescence of fever after 36 hours of immunoglobulin therapy, it has a prevalence of just 10-15%, barely 2 -5 per 100,000 – which is how rare this is! The risk to the heart is even greater in this group, but with such a low incidence, there is lack of high quality evidence of optimal management. Other treatments considered are steroids or iniximab. Eosinophila is a known factor in resistance (as was in our patient) with predictive poor response to treatment, though happily, I. responded to the second course of IV Ig. In this era of covid, autoimmune phenomenon & MISC like disease (multisystem inammatory disease in children) is being increasingly seen.
Take-home message: early diagnosis and treatment of Kawasaki is crucial to prevent complications which can have life long implications for a child, especially to the heart. Children with this potentially dangerous disease can land in the treatment hands of any speciality like for lymphadenopathy to the ENT department , to the dermatologist for erythroderma or psoriatic like rash or even to the surgeon with an acute abdomen.
The possibility of such a disease should be in the back of everyone’s mind when diagnosis is unclear and the patient is not responding to the usual lines of management.