Adult-onset Still’s disease: rare cause for prolonged fever syndrome

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Omar El-Mourad1, Fabien Roodhans1,2, Valérie Huyge3 Published in the journal : April 2019 Category : Observations cliniques

Summary :

The adult-onset Still’s disease is a rare cause of fever of unknown origin. The clinical presentation and biological signs are rather polymorphous and non-specific. We herein emphasize the usefulness of measuring serum ferritin and glycosylated ferritin (GF) levels. The diagnosis of adult-onset Still’s disease is based on the Yamaguchi criteria, with three major criteria (high fever for more than 1 week, neutrophilic leucocytosis, and diffuse arthralgia) and three minor criteria (liver enzyme elevation of neither toxic nor drug-related origin, lymphadenopathy identified on PET imaging, and negativity of rheumatoid factors and antinuclear antibodies) to be present. The disease’s pathophysiology is still uncertain, and treatment essentially relies on systemic glucocorticoids. In this paper, we have discussed the usefulness of biotherapies, comprising primarily the monoclonal antibodies directed against the IL-6 receptor.

What is already known about the topic?

The diagnosis of adult-onset Still’s disease remains a difficult challenge, owing to the lack of specific clinical and biological signs. It is, thus, a diagnosis of exclusion after elimination of other differential diagnoses, such as infectious, inflammatory, and neoplastic diseases. Glucocorticoids are still key to a successful treatment.

What does this article bring up for us?

The article discusses the usefulness of measuring ferritin and glycosylated ferritin levels. In addition to glucocorticoids, biotherapies have been proven to be increasingly effective and well-tolerated.

Key Words

Fever, musculo-skeletal pain, lymphadenopathy, inflammatory syndrome, glucocorticoids