Adult Onset Still’s Disease - Case Report of 2 Cases

Abstract

Adult onset Still’s disease (AOSD) is a rare disease in adults, in children also known as systemic juvenile idiopathic arthritis. We describe two patients with intermittent fevers without unknown origin. 27 years man and 75 years old woman, who presented with lymphadenopathy and recurrent fevers, There has been used intensive serologic, radiologic, laboratory investigation to exclude infectious diseases and malignancy. All the investigation showed no diagnosis. The clinical disease described for the first time 105 years ago by Dr Still is finally diagnosed. Both patients received Anakinra with rapid response in hematologic, biochemical, and cytokine markers with reduction of systemic and local inflammation. Adult-onset Still’s disease is a rare systemic inflammatory disease of unknown etiology that often presents as a fever of unknown origin. Systemic features, such as spiking fever, skin rash, generalized lymphadenopathy, hepatomegaly, splenomegaly, and serositis, was first described by the British paediatrician Still [1]. The aetiology of AOSD still remains unknown, but over expression of Th1 cytokines and IL-1 may have a critical role [2]. Daily fevers, evanescent rash, arthritis, hyperferritinemia, and liver dysfunction were consistent with AOSD. Hyperferritinemia has a high sensitivity for AOSD (80%) but has a low specificity (40%). The fraction of glycosylated ferritin is higher than with inflammatory conditions. The combination of elevated serum ferritin level and low (20%) fraction of glycosylated ferritin can make the diagnosis of AOSD most likely. In fact AOSD has similarities to auto inflammatory diseases, as exemplified by a central role of the innate immune system and by the cytokines involved (e.g., interleukin-1 [IL-1]). Moreover, the blocking of both IL-1 has been shown to be efficacious in the treatment of AOSD. An interleukin-1 (IL-1) antagonism, e.g., with the IL-1 receptor antagonist Anakinra, is the standard of therapy for the exacerbations of acute disease rather than increasing steroid use.