Atypical Kawasaki Disease

Abstract

The child was dull-looking, irritable and dehydrated. She had tachycardia and tachypnoea but no chest findings. The cardiac exam was normal. Initial diagnosis of Acute Gastroenteritis with some dehydration was made. Relevant investigations were sent, which showed high CRP, hence, Inj. Ceftriaxone were added and dehydration was corrected along with supportive measures. In view of irritability and high CRP, Lumbar Puncture was advised, but the parents refused. wStool analysis was normal and negative for reducing sugar and rotavirus antigen. With the above measures, the child initially showed improvement in lab parameters. Blood culture reported sterile, but fever spikes persisted and again on day 6, increased CRP, with a persistent dull appearance; antibiotics were upgraded to Inj. Meropenem and Amikacin. After 48 hours of upgradation of antibiotics, fever and irritability were persistent. A Neurology opinion was taken, an MRI of the Brain and EEG and a CSF examination was done, which showed no sequelae of Meningoencephalitis. As fever spikes continued, autoimmune causes of fever were considered and inflammatory markers (LDH and Ferritin), within normal range, were sent. 2D ECHO (Figure-1) was done on day 8 of illness, which showed dilated coronaries suggestive of Kawasaki Disease Z scores LM- 5.5, LAD- 6 RCA-5.2. IVIG 2gm/kg over 24 hrs along with Aspirin as per the dose recommended by the Paediatric Cardiologist was given. As the Fever persisted, despite IVIG infusion at 48 hrs, repeat CBC and CRP were done, which showed reduced CRP and improved TLC. 2D ECHO was repeated on day 10 of illness, which showed a further increase in Z-score in dilatation of coronaries (Z scores- LM +6.46. LAD- +10, RCA-+9.1). So, a repeat dose of IVIG along with IV steroids and LMWH was given and the child was shifted to a dedicated cardiac monitoring centre under Paediatric Rheumatologist. Gradually, fever spikes settled on repeat 2D ECHO, Z-score was further increased LM- 5.28, LAD-11.98, RCA-9.2, so started on cyclosporine and steroid continued. On follow-up, a week later, a gradual decrease in LMCA 2.5, LAD- 3.2 and RCA 3.9 in coronary dilatation was seen.