P163 The cytokine horror: Kawasaki disease shock syndrome complicated with macrophage activation syndrome

Abstract Background/Aims Kawasaki disease (KD) is an acute febrile systemic vasculitis of unknown aetiology, affecting medium-sized vessels. It often occurs in children less than 5 years old. During the phase disease, 5% develop hypotension and shock. This severe form called Disease Shock Syndrome (KDSS). Macrophage activation syndrome (MAS) a heterogenous complex encompassing broad spectrum clinical features. MAS associated with KDSS extremely rare life threatening entity that warrants high index suspicion, prompt proper management. Methods We present our experience dilemmas challenges faced diagnosis management complicated poor resource setting. first encountering KDSS. Results A 9-month-old baby fever maculopapular rash involving palms soles for days duration was transferred from peripheral hospital. preliminary made classical features included rash, cracked lips, pedal oedema, BCG scar inflammation, perianal excoriation irritability. The did not settle following standard therapy three doses intravenous immunoglobulins 2g/kg, aspirin low dose dexamethasone. child quickly began to decompensate. She developed cold clammy skin on 8th day illness. There evidence hepatosplenomegaly transaminitis. 2D Echo revealed normal coronary vasculature without any dilatations or strictures. However, there myocarditis supported by diffuse T inversions anterolateral leads highly positive troponin titre. Investigations aa leucocytosis (22,100/dl), abrupt drop platelet count (350,000/dl 80,000/dl), red blood cells, haemoglobin (11g/dl 8g/dl) erythrocyte sedimentation rate 95mm/hr 50mm/hr. Hyperferritinemia, hypertriglyceridemia hypofibrinogenemia MAS. Blood culture sterile. Initial bone marrow biopsy inconclusive. High dexamethasone 10mg/m2/day cyclosporine 2mg/kg/day initiated. circulatory collapse managed fluid boluses adrenaline infusion. Subsequently dramatic improvement her cell counts transaminases normalised 15. Conclusion emphasise importance early recognition refractory pediatric populations rheumatic diseases we suggest initiation cyclosporin as rapid effective treatment option settings where ideal anakinra beyond realms possibility. Disclosure L. Rajagopala: None. J. Jagoda: M.L. Gunatilaka: