Case report
Pulmonary arterial hypertension secondary to adult-onset Still’s disease: Response to cyclosporine and sildenafil over 15 years of follow-up

https://doi.org/10.1016/j.rmcr.2016.06.007Get rights and content
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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.

Keywords

Pulmonary arterial hypertension
Still’s disease
Adult onset
Cyclosporine
Sildenafil

Abbreviations

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

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