Still´s disease – a rare autoinflammatory illness of adults: case report and overview of therapeutic options

Czech version

Authors: Z. Král ¹; M. Krejčí ¹; Z. Řehák ²; R. Koukalová ²; L. Pour ¹; M. Krtička ³; Z. Adam ¹
Authors‘ workplace: Interní hematologická a onkologická klinika LF MU a FN Brno ¹; Oddělení nukleární medicíny, MOU Brno ²; Klinika úrazové chirurgie LF MU a FN Brno ³
Published in: Transfuze Hematol. dnes,26, 2020, No. 4, p. 320-326.
Category: Case Reports

Overview

Still's disease (Adult-onset Still's disease, AODS) is a very rare systemic inflammatory disease accompanied by a triad of spiking fever, maculopapular exanthema and arthralgia. To date, there is no specific laboratory or imaging test available for AODS. We report the case of a 65-year-old male who suffered from fever and arthralgia. Initial laboratory tests showed elevated CRP, leukocytosis and anaemia. Metabolically active abdominal lymphadenopathy was present. We excluded many potential differential diagnoses and the diagnosis of lymphoma was not confirmed. After an extensive workup, the patient was diagnosed with AOSD based on the Yamaguchi criteria. The first line therapy was prednisone at a dose of 1mg/kg, after which all AODS symptoms disappeared including fever. After tapering the prednisone dose, the AOSD relapsed. The patient then received anakinra, 100 mg daily as second line treatment and achieved a good response, but it was necessary to continue this treatment. Our patient has now been treated with anakinra from 2016-to date and remains asymptomatic. In this article, we describe our AOSD case and provide a brief overview of typical AOSD clinical symptoms and treatment options.

Keywords:

adult-onset Still's disease (AOSD) – anakinra

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