Refractory Adult-Onset Still’s Disease: A Case Report

It is often a challenge and a dilemma for clinicians encountering patients with pyrexia of unknown origin. Numerous tests performed to determine the underlying cause often give inconclusive results. We present a 52-year-old man with undulating fever for more than ten months consistent with diagnosis of pyrexia of unknown origin (PUO).  It was associated with persistent hyperferritinaemia, elevated C-reactive protein (CRP) and erythrocyte sedimentation rate (ESR), leukocytosis, thrombocytopaenia, hepatosplenomegaly, hypertrigyceridaemia, raised fibrinogen index and arthritis of the ankle joints. Serological markers, repeated septic workout, cerebrospinal fluid and bone marrow and trephine biopsy (BMT) studies were all negative otherwise.  The imaging studies (computed tomography scan of whole body) apart from findings of mild hepatosplenomegaly, was otherwise normal. The positron-emission tomography (PET) scan shows evidence of polyarthritis. Based on these findings, diagnosis of refractory adult-onset Still’s disease complicated by haemophagocytic lymphohistiocytosis was made although the BMT findings was inconclusive. Despite corticosteroids, disease modifying anti-rheumatic agents (DMARD) and immune-modulator therapy, he succumbed to the illness.  This case history illustrates the diagnostic complexity of adult-onset Still’s disease with protean manifestation.