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Case Reports in Rheumatology
Volume 2012 (2012), Article ID 537613, 5 pages
http://dx.doi.org/10.1155/2012/537613
Case Report

Pulmonary Arterial Hypertension in Adult-Onset Still’s Disease: Rapid Response to Anakinra

Division of Rheumatology, University of Michigan, P.O. Box 481, Ann Arbor, MI 48109, USA

Received 30 April 2012; Accepted 12 June 2012

Academic Editors: R. Aminov and S. Hamoud

Copyright © 2012 Marc Campos and Elena Schiopu. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Adult-onset Still’s disease (AOSD) is a rare inflammatory condition characterized by spiking quotidian fever, rash, chronic arthralgia, leukocytosis, and occasional pulmonary involvement such as pleural effusion and transient pulmonary infiltrates. Pulmonary arterial hypertension (PAH) is a rare pulmonary complication of AOSD, and we are aware of only 5 cases reported in the literature. We report the case of a 27-year-old woman of Middle Eastern descent, with a 7-year history of AOSD, who developed severe pulmonary arterial hypertension (PAH). After unsuccessful exposure to various immunosuppressive regimens, shortly following the initiation of anakinra, an interleukin-1 (IL-1) receptor antagonist, her disease became quiescent and the PAH resolved. With this case report, we hope to show that anakinra, either by virtue of controlling the overall inflammation in AOSD, or by direct effect on the pulmonary microangiopathy, can improve severe PAH.