title: Sildenafil improves walk distance in idiopathic pulmonary fibrosis.
abstract: Pulmonary hypertension is a common finding in patients with idiopathic pulmonary fibrosis (IPF), and is associated with increased morbidity and mortality. Therapy with sildenafil has been shown to decrease pulmonary vascular resistance in patients with pulmonary fibrosis and may improve functional status. Patients with IPF and documented pulmonary hypertension were followed up in an open-label study of sildenafil. The 6-min walk test distance (6MWD) was obtained before and after 3 months of sildenafil therapy. Fourteen patients were followed up in the study; 11 patients completed both 6-min walk tests. The mean improvement in walk distance was 49.0 m (90% confidence interval, 17.5 to 84.0 m). When all 14 patients were dichotomized into groups of "responders" (ie, >/= 20% improvement in 6MWD) or "nonresponders" (ie, < 20% change or unable to complete), 57% were classified as responders. Sildenafil is a promising and well-tolerated therapeutic agent for use in patients with IPF and pulmonary hypertension, and should be studied in a large, well-controlled trial.
authors.affiliation: Department of Medicine, San Francisco General Hospital, University of California San Francisco, San Francisco, CA.
authors.forename: Harold R
authors.initials: HR
authors.lastname: Collard
authors.affiliation: Duke Clinical Research Institute, Duke University, Durham, NC.
authors.forename: Kevin J
authors.initials: KJ
authors.lastname: Anstrom
authors.affiliation: Department of Medicine, University of Colorado Health Sciences Center, Denver, CO.
authors.forename: Marvin I
authors.initials: MI
authors.lastname: Schwarz
authors.affiliation: Department of Medicine, David Geffen School of Medicine, University of California Los Angeles, Los Angeles, CA. Electronic address: dzisman@mednet.ucla.edu.
authors.forename: David A
authors.initials: DA
authors.lastname: Zisman
mesh_terms: D015106:3',5'-Cyclic-GMP Phosphodiesterases / Q000037:antagonists & inhibitors*; D000368:Aged; D000369:Aged, 80 and over; D054706:Cyclic Nucleotide Phosphodiesterases, Type 5; D005080:Exercise Test / Q000187:drug effects*; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D006976:Hypertension, Pulmonary / Q000188:drug therapy*; D008297:Male; D008875:Middle Aged; D010726:Phosphodiesterase Inhibitors / Q000627:therapeutic use*; D010879:Piperazines / Q000627:therapeutic use*; D011658:Pulmonary Fibrosis / Q000188:drug therapy*; D011669:Pulmonary Wedge Pressure / Q000187:drug effects; D011687:Purines / Q000627:therapeutic use; D000068677:Sildenafil Citrate; D013450:Sulfones / Q000627:therapeutic use*; D014655:Vascular Resistance / Q000187:drug effects; D014665:Vasodilator Agents / Q000627:therapeutic use*; D016138:Walking
chemical_list: D010726:Phosphodiesterase Inhibitors; D010879:Piperazines; D011687:Purines; D013450:Sulfones; D014665:Vasodilator Agents; D000068677:Sildenafil Citrate; D015106:3',5'-Cyclic-GMP Phosphodiesterases; D054706:Cyclic Nucleotide Phosphodiesterases, Type 5; C517381:PDE5A protein, human