Abstract
We report the case of a patient with severe adult-onset still's disease not responding to treatment with high-dose corticosteroids and intravenous immunoglobulins. Partial remission was obtained under therapy associating Anakinra and Leflunomide but subsequently she developed acute hepatitis and disseminated intravascular coagulation consistent with a reactive Hemophagocytic lymphohistiocytosis. The patient was successfully treated with a combination of Tocilizumab, Cyclosporine and corticosteroids leading to a prolonged remission. This report illustrates the difficulty to treat adult-onset Still's disease complicated by Hemophagocytic lymphohistiocytosis and the usefulness of liver biopsy to investigate acute hepatitis in this clinical setting.
Highlights
Adult onset Still’s disease (AOSD) is a rare inflammatory disorder that mainly affects young adults with a slight preponderance for women [1]
The precise etiology and pathogenesis of this disorder remain poorly defined, but it is suggested that the inappropriate production of IL-1 and other pro-inflammatory cytokines, including TNF, IL-6 and IL-18 may play a role in genetically predisposed hosts
CsA was stopped after 5 months, prednisone tapered to 5 mg/d, and monthly tocilizumab continued. This case report illustrates the challenge in the diagnosis and treatment of AOSD
Summary
Adult onset Still’s disease (AOSD) is a rare inflammatory disorder that mainly affects young adults with a slight preponderance for women [1]. Adult onset Still’s disease, Macrophage activation syndrome, Reactive hemophagocytic lymphohistiocytosis, Hyperferritinemic syndrome, Acute liver injury, Cyclosporine A, Tocilizumab, Anakinra, High dose immunoglobulins The distinction between severe AOSD and HLH may be difficult, since they share common features, especially hepatic injury.
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