Pulmonary arterial hypertension secondary to adult-onset Still's disease: Response to cyclosporine and sildenafil over 15 years of follow-up. (2016)

• Record Type: Journal Article
• Authors: Weatherald, Jason; Lategan, Johan; Helmersen, Doug
• Is Part Of: Respiratory medicine case reports. Volume 19(2016)
• Page Start: 27
• Page End: 30
• Publication Date: 2016
• Subjects: Pulmonary arterial hypertension -- Still's disease -- Adult onset -- Cyclosporine -- Sildenafil; AOSD adult onset Still's disease -- PAH pulmonary arterial hypertension -- ANA antinuclear antibody -- RF rheumatoid factor -- SLE systemic lupus erythematosus -- RVSP right ventricular systolic pressure -- IL interleukin -- WHO World Health Organization -- RHC right heart catheterization -- mPAP mean pulmonary arterial pressure -- PVR pulmonary vascular resistance -- 6MWD 6-minute walk distance -- PH pulmonary hypertension -- NFAT nuclear factor of activated T cells; Respiratory organs -- Diseases -- Periodicals; Chest -- Diseases -- Periodicals; Respiratory Tract Diseases -- Periodicals; Respiratory System -- Periodicals; Chest -- Diseases; Respiratory organs -- Diseases; Electronic journals; Periodicals; 616.2
• DOI: 10.1016/j.rmcr.2016.06.007
• Languages: English
• ISSNs: 2213-0071

Abstract

Abstract: Adult onset Still's disease (AOSD) is an autoimmune disease characterized by systemic inflammation and is a rarely reported cause of pulmonary arterial hypertension (PAH). We describe the clinical course of a 40-year-old woman who presented with PAH 19 months after a diagnosis of AOSD. Sildenafil and immunosuppressive therapy with cyclosporine resulted in clinical and hemodynamic improvement with long-term survival 15 years after her initial presentation of AOSD. We review the literature for published cases of PAH due to AOSD and discuss the potential mechanisms relating inflammatory diseases and PAH.