title: Successful treatment with sildenafil in systemic sclerosis patients with isolated pulmonary arterial hypertension: two case reports.
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.
authors.affiliation: Department of Dermatology, Kanazawa University Graduate School of Medical Science , 13-1 Takaramachi, Kanazawa, Ishikawa 920-8641, Japan.
authors.forename: Ikuko
authors.initials: I
authors.lastname: Hayakawa
authors.forename: Fumiaki
authors.initials: F
authors.lastname: Shirasaki
authors.forename: Takashi
authors.initials: T
authors.lastname: Hirano
authors.forename: Naoto
authors.initials: N
authors.lastname: Oishi
authors.forename: Minoru
authors.initials: M
authors.lastname: Hasegawa
authors.forename: Shinichi
authors.initials: S
authors.lastname: Sato
authors.forename: Kazuhiko
authors.initials: K
authors.lastname: Takehara
mesh_terms: D005260:Female; D006439:Hemodynamics / Q000187:drug effects; D006801:Humans; D006976:Hypertension, Pulmonary / Q000188:drug therapy* / Q000209:etiology; D008875:Middle Aged; D020097:Natriuretic Peptide, Brain / Q000097:blood; D010879:Piperazines / Q000008:administration & dosage*; D011687:Purines; D012595:Scleroderma, Systemic / Q000150:complications*; D000068677:Sildenafil Citrate; D013450:Sulfones; D014665:Vasodilator Agents / Q000008:administration & dosage*
chemical_list: D010879:Piperazines; D011687:Purines; D013450:Sulfones; D014665:Vasodilator Agents; D020097:Natriuretic Peptide, Brain; D000068677:Sildenafil Citrate