Smoking-related interstitial fibrosis (SRIF) and pulmonary hypertension

• Published in: BMJ case reports Vol. 2013; p. bcr2013008970
• Main Authors: El-Kersh, Karim, Perez, Rafael L, Smith, J Shaun, Fraig, Mostafa
• Format: Journal Article
• Language: English
• Published: England BMJ Publishing Group LTD 11.03.2013; BMJ Publishing Group
• Series: Case Report
• Subjects: Antihypertensive Agents - therapeutic use; Biopsy; Carbolines - therapeutic use; Cardiac Catheterization; Drug Therapy, Combination; Humans; Hypertension, Pulmonary - diagnosis; Hypertension, Pulmonary - drug therapy; Hypertension, Pulmonary - etiology; Lung diseases; Male; Middle Aged; Pulmonary arteries; Pulmonary Fibrosis - diagnosis; Pulmonary Fibrosis - drug therapy; Pulmonary Fibrosis - etiology; Pulmonary hypertension; Respiratory Function Tests; Smoking; Smoking - adverse effects; Sulfonamides - therapeutic use; Tadalafil; Thromboembolism; Tomography, X-Ray Computed; Unusual Association of Diseases/Symptoms; USA/Canada; Vasodilator Agents - therapeutic use; Veins & arteries; Ventilation; White
• ISSN: 1757-790X; 1757-790X
• DOI: 10.1136/bcr-2013-008970

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.