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Abstract
A 9 week old baby presented with fever and irritability. A septic screen was negative, though CSF showed lymphocytic pleocytosis. She developed marked peripheral ischaemia and focal necrotic tips of fingers and toes. Echocardiogram was normal (including coronary arteries). Raised inflammatory markers, pyrexia and the vascular concerns suggested vasculitis and initial management was with epoprostenol and high dose methylprednisolone and aspirin. With improved perfusion focal fingertip necrosis developed. US and later MR angiogram showed bilateral brachial artery aneurysms with precarious thrombi with risk of further embolisation. In view of continuing inflammation IV immunoglobulin and then infliximab were given which switched off the inflammatory process.
Following a nervous period of observation, good control of inflammation allowed the possibility of excision of both aneurysms, with vein grafts on both sides which took place without complication. Subsequently excellent progress has been made with development and growth, with no evidence of return of inflammation at 4 months. Despite no coronary artery concerns and none of the diagnostic criteria for Kawasaki disease (except for pyrexia) histology and subsequent progress strongly points to the diagnosis of Kawasaki disease.
Learning points
▶ In an infant with persistent fever, not responding to antibiotics, especially if irritable, Kawasaki disease must be considered even if classical diagnostic criteria are not met
▶ Aseptic meningitis can be seen in autoimmune connective tissue diseases.
▶ Vasculitis process in Kawasaki's disease can affect small and medium vessel arteries (including with aneurysm) although coronary artery aneurysms are most commonly described. Emboli can develop from these aneurysms and due to the vasculitis process, and should be considered if any peripheral ischaemia / necrosis.