Adult onset still’s disease; a rare disease in Nigeria?

Abstract

Adult Onset Still’s Disease (AOSD) is an inflammatory disease of unknown aetiology. Its global prevalence is estimated at 1 case per 100,000. Because of its pattern of presentation which mimics many inflammatory and malignant conditions, the diagnosis requires high index of suspicion. Few cases have been reported from Africa. The first case in Nigeria was reported in July 2015. We hereby report two more cases diagnosed in the same rheumatology clinic of Lagos State University Teaching Hospital within six months of the first reported case. This is to highlight the fact that the disease while rare, requires a high index of suspicion for diagnosis. Both patients were males. The ages of the patients were 19 and 62 years. Both patients had high grade fever, symmetrical inflammatory polyarthritis and weight loss. The first patient had sore throat. On examination, both were found to be febrile. The second was emaciated and pale. Both patients had marked leukocytosis with neutrophil predominance, thrombocytosis, elevated liver enzymes and elevated acute phase rectants. Rheumatoid factor, anti-CCP, anti-nuclear antibody and extractable nuclear antigen were negative in both patients. Serum ferritin was markedly elevated in both. Retroviral screening, anti-HCV and HBsAg were negative in both. Septic work up and direct Coomb’s test were negative in them. Peripheral blood film was normal and bone marrow aspirate was suggestive of chronic inflammatory condition in the second patient. The first patient was treated with steroid, hydroxychloroquine and azathioprine. The second patient was treated with steroid and methotrexate. Both made good clinical recovery.