Open AccessAn Atypical Presentation of Adult‐Onset Still's Disease Complicated by Pulmonary Hypertension and Macrophage Activation Syndrome Treated with Immunosuppression: A Case‐based Review of the Literature
Author(s) -
Mehta Mili V.,
Manson Daniel K.,
Horn Evelyn M.,
Haythe Jennifer
Publication year - 2016
Publication title -
pulmonary circulation
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.791
H-Index - 40
ISSN - 2045-8940
DOI - 10.1086/685112
Subject(s) - medicine , macrophage activation syndrome , anakinra , immunosuppression , pulmonary hypertension , adult onset still's disease , disease , presentation (obstetrics) , complication , dermatology , surgery
Pulmonary arterial hypertension (PAH) is a known complication of rheumatologic diseases, but it is only rarely associated with adult‐onset Still's disease (AOSD). We describe the case of a 30‐year‐old woman who presented in a pulmonary hypertension crisis and was found to have underlying AOSD with PAH and nonspecific interstitial pneumonia (NSIP) with a course complicated by macrophage activation syndrome (MAS). She dramatically improved with steroids, cyclosporine A, and anakinra, with total resolution of the MAS and significant improvement of her pulmonary arterial pressures. While there are only select case reports of AOSD associated with PAH, this is the first reported case of (1) AOSD complicated by both PAH and MAS and (2) AOSD complicated by biopsy‐proven NSIP. Clinically, this case highlights the efficacy of immunosuppressive agents in the treatment of PAH and MAS from underlying AOSD and supports their use in this setting.