Adult-onset Still’s disease in a 73-year-old Maltese man

Abstract

Adult-onset Still’s disease (AOSD) is a rare autoinflammatory condition diagnosed by Yamaguchi criteria. We report an atypical presentation of a 73-year-old man, who was admitted with fever, sore throat and pleurisy that were unresponsive to three courses of antibiotics. Fever persisted in a quotidian pattern and the typical salmon-coloured rash consistent with AOSD appeared at 4 weeks from symptoms onset. These features in addition to neutrophilia and hyperferritinaemia in the absence of concurrent infectious and neoplastic causes satisfied Yamaguchi criteria for a diagnosis of AOSD. Epstein-Barr antigen was initially detected at moderate titre levels, but was undetectable after 1 week. Complete resolution of symptoms was reported on initiation of steroid treatment. A relationship between disease onset and viral syndromes has been documented to occur and few similar cases preceded by Epstein-Barr virus detection have been reported.