An 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

PVRI Member Authors: Evelyn Horn


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.

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Immunology/ Immune Cell Signaling/ Inflammation/ Immunotherapy
Inflammatory and Immune Cells
Monocytes/ Macrophages/ Neutrophils/ Dendritic Cells/ Mast Cells
Pulmonary Arterial Hypertension


Mili V. Mehta, Daniel K. Manson, Evelyn M. Horn, Jennifer Haythe

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Pulmonary Circulation Vol 6: No 1 cover image

March 2016

Pulmonary Circulation Vol 6: No 1

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