Successful treatment with sildenafil in systemic sclerosis patients with isolated pulmonary arterial hypertension: two case reports - PubMed (original) (raw)
Case Reports
Successful treatment with sildenafil in systemic sclerosis patients with isolated pulmonary arterial hypertension: two case reports
Ikuko Hayakawa et al. Rheumatol Int. 2006 Jan.
Abstract
We describe two systemic sclerosis (SSc) patients with isolated pulmonary arterial hypertension (PAH) who were given treatment with 50 mg oral sildenafil per day. We evaluated the efficacy of oral sildenafil for isolated PAH in SSc patients by direct assessment with cardiac catheterization before and 6 months after the initiation of sildenafil. Right-heart catheterization demonstrated decreased mean pulmonary artery pressure, decreased pulmonary vascular resistance, and increased cardiac output after treatment with sildenafil. Brain natriuretic peptide levels were gradually decreased. The 6-min walking distance was greatly extended. Moreover, the physical conditions of both patients were much improved. We recognized no adverse events. We propose that oral sildenafil may be beneficial as a selective pulmonary vasodilator and as long-term treatment in SSc patients with isolated PAH.
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