Adult-onset Still’s disease – a difficult path to diagnosis through fever and effusions of unknown origin

Česká verzia

Authors: Jana Malegová ¹; Lukáš Koten ¹; Pavel Horák ²
Authors place of work: Interní klinika IPVZ Praha a Krajské nemocnice T. Bati, a. s., Zlín, přednosta prim. MUDr. Jiří Latta ¹; III. interní klinika - nefrologie, revmatologie a endokrinologie LF UP a FN Olomouc, přednosta prof. MUDr. Josef Zadražil, CSc. ²
Published in the journal: Vnitř Lék 2014; 60(5-6): 520-526
Category: Case Report

Summary

Fever of unknown origin, pleural and pericardial effusions can be caused by a variety of independent agents. On the other hand, we can identify a common causative condition in other cases. Infectious diseases, malignancies and autoimmune diseases are the most common etiological factors. Considering the pleural and pericardial effusion, we also have to think of cardiovascular and pulmonary diseases. The basis of every diagnostic process is thorough medical history and detailed clinical examination followed by laboratory and imaging methods. In spite of that, the right diagnosis sometimes stays long time hidden. One of such conditions is Adult-onset Still’s disease (AOSD). It is a rare inflammatory, potentially life-threatening disease with unclear pathogenesis and heterogeneous symptoms. It has some features similar to systemic form of juvenile idiopathic arthritis. Diagnosis is established so called per exclusionem by fulfilling a set of clinical criteria and ruling out other diseases with similar symptomatology. In our article, we present an example of such an arduous diagnostic journey to final diagnosis.

Key words:
anakinra – arthralgia – evanescent rash – ferritin – fever of unknown origin – pericardial effusion – pleural effusion – procalcitonin – Still’s disease

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