title: Smoking-related interstitial fibrosis (SRIF) and pulmonary hypertension.
abstract: Smoking-related interstitial fibrosis (SRIF) is a relatively new term used to describe chronic interstitial fibrosis that can develop in smokers. The association of SRIF with pulmonary hypertension has not been described. We present a 55-year-old man with an extensive smoking history who presented for evaluation of insidious onset of dyspnoea on exertion and hypoxaemic respiratory failure. Physical examination was unremarkable. Pulmonary function testing demonstrated a marked reduction of the diffusion capacity with no obstruction or restriction. Ventilation perfusion scan showed no evidence of thromboembolic disease. High-resolution chest CT revealed minimal biapical pleural parenchymal scarring and subtle dependent atelectasis. Serological markers for connective tissue diseases were negative. Open lung biopsy was consistent with SRIF. Vascular intimal proliferation consistent with pulmonary hypertension was also noted. Right heart catheterisation yielded mild pulmonary hypertension and treatment was initiated with tadalafil and bosentan.
authors.affiliation: Department of Pulmonary, Critical Care & Sleep Medicine, University of Louisville, Louisville, Kentucky, USA. karimelkersh@yahoo.com
authors.forename: Karim
authors.initials: K
authors.lastname: El-Kersh
authors.forename: Rafael L
authors.initials: RL
authors.lastname: Perez
authors.forename: J Shaun
authors.initials: JS
authors.lastname: Smith
authors.forename: Mostafa
authors.initials: M
authors.lastname: Fraig
mesh_terms: D000959:Antihypertensive Agents / Q000627:therapeutic use; D001706:Biopsy; D000077300:Bosentan; D002243:Carbolines / Q000627:therapeutic use; D006328:Cardiac Catheterization; D004359:Drug Therapy, Combination; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology*; D008297:Male; D008875:Middle Aged; D011658:Pulmonary Fibrosis / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology*; D012129:Respiratory Function Tests; D012907:Smoking / Q000009:adverse effects*; D013449:Sulfonamides / Q000627:therapeutic use; D000068581:Tadalafil; D014057:Tomography, X-Ray Computed; D014665:Vasodilator Agents / Q000627:therapeutic use
chemical_list: D000959:Antihypertensive Agents; D002243:Carbolines; D013449:Sulfonamides; D014665:Vasodilator Agents; D000068581:Tadalafil; D000077300:Bosentan