Complications of adult-onset Still's disease and their management
- PMID: 29658384
- DOI: 10.1080/1744666X.2018.1465821
Complications of adult-onset Still's disease and their management
Abstract
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; biological agents; disseminated intravascular coagulation; hepatitis; interleukin-1 inhibitors; interleukin-6 inhibitor; pericarditis; pulmonary arterial hypertension; reactive hemophagocytic lymphohistiocytosis; thrombotic microangioapathy.
Comment in
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Thrombotic microangiopathy in adult-onset Still's disease: the story is just beginning.Expert Rev Clin Immunol. 2019 Nov;15(11):1123-1124. doi: 10.1080/1744666X.2019.1682892. Epub 2019 Oct 31. Expert Rev Clin Immunol. 2019. PMID: 31650884 No abstract available.
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