title: Pulmonary arterial hypertension secondary to adult-onset Still's disease: Response to cyclosporine and sildenafil over 15 years of follow-up.
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.
authors.affiliation: Division of Respirology, Department of Medicine, University of Calgary, Calgary, Alberta, Canada.
authors.forename: Jason
authors.initials: J
authors.lastname: Weatherald
authors.affiliation: Division of Hematology, Department of Medicine, University of Calgary, Calgary, Alberta, Canada.
authors.forename: Johan
authors.initials: J
authors.lastname: Lategan
authors.affiliation: Division of Respirology, Department of Medicine, University of Calgary, Calgary, Alberta, Canada.
authors.forename: Doug
authors.initials: D
authors.lastname: Helmersen
keywords: 6MWD, 6-minute walk distance; ANA, antinuclear antibody; AOSD, adult onset Still’s disease; Adult onset; Cyclosporine; IL, interleukin; NFAT, nuclear factor of activated T cells; PAH, pulmonary arterial hypertension; PH, pulmonary hypertension; PVR, pulmonary vascular resistance; Pulmonary arterial hypertension; RF, rheumatoid factor; RHC, right heart catheterization; RVSP, right ventricular systolic pressure; SLE, systemic lupus erythematosus; Sildenafil; Still’s disease; WHO, World Health Organization; mPAP, mean pulmonary arterial pressure