Complications of adult-onset Still’s disease and their management

ABSTRACT

Introduction: Adult-onset Still’s disease (AOSD) is a rare systemic auto-inflammatory disorder in which management and treatment have considerably progressed over the past decade. Despite wide use of interleukin (IL)-1 or IL-6 inhibitors, serious complications remain possible.

Areas covered: A comprehensive literature search in MEDLINE via Pubmed was performed to review AOSD’s severe and sometimes life-threatening complications: reactive hemophagocytic lymphohystiocytosis, coagulation disorders, fulminant hepatitis, cardiac or pulmonary complications and amyloid A amyloidosis.

Expert commentary: Early recognition and prompt management is essential to significantly decrease morbi-mortality. The key question is to determine whether the complication is related to the disease itself or related to or favored by (e.g. infection) the ongoing treatment.

For all severe AOSD-related complications, high-dose corticosteroids and supportive measures remain the first-line treatment. In case of inadequate response, combination with IL-1 or IL-6 blockers is justified. Cyclosporine A and etoposide remain of interest, especially in case of reactive hemophagocytic lymphohysitocytosis. Plasma exchange may be useful in case of thrombotic microangiopathy. In the near future, new biologic or non-biologic drugs targeting IL-18 or other cytokines or kinases could be of help.

KEYWORDS:

• Adult-onset Still’s disease
• reactive hemophagocytic lymphohistiocytosis
• disseminated intravascular coagulation
• thrombotic microangioapathy
• hepatitis
• pericarditis
• pulmonary arterial hypertension
• biological agents
• interleukin-1 inhibitors
• interleukin-6 inhibitor

Declaration of interest

B Fautrel has received consultant fees from AbbVie, Biogen, BMS, Celgene, Medac, MSD, Nordic, Novartis, Pfizer, Roche, Sanofi-Aventis, SOBI, UCB, and research grants from AbbVie, MSD and Pfizer. The authors have no other relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript apart from those disclosed. Peer reviewers on this manuscript have no relevant financial or other relationships to disclose.

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This article was not funded.