Case reportPulmonary arterial hypertension secondary to adult-onset Still’s disease: Response to cyclosporine and sildenafil over 15 years of follow-up
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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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© 2016 The Authors. Published by Elsevier Ltd.