Abstract
Background: Systemic sclerosis is a connective tissue disorder, manifesting as skin thickening and multi organ involvement. Scleroderma lung disease is the leading cause of death in this entity. The most common pulmonary presentations are interstitial lung disease and pulmonary hypertension,
Objectives: We investigated different presentations of lung disease in scleroderma patients in Masih Daneshvari hospital between October 2000 and September 2009 in a retrospective study and clinical and paraclinical results were reported.
Results: 42 patients were identified with scleroderma lung disease. The majority of the patients (84%) were female and 23% were male with mean age of 47.26±14.30. The most common complain was dyspnea followed by cough and orthopnea in 93%, 69% and 19% of the patients respectively. Echocardiography demonstrated elevated pulmonary artery pressure (≥30 mmhg) in 17 patients (40.4%). Association of pneumothorax and adenocarcinoma was noted in 3 and 1 of the patients respectively. Finally, 6 patients were deceased and 3 patients are expecting lung transplantation due to advanced lung fibrosis.
HRCT findings
Conclusion: This study from a tertiary referral hospital in Iran illustrates similar results of pulmonary involvements in systemic sclerosis patients in comparison with previous reports.
- © 2011 ERS
