Survival in CVD-ILD at Ege University Hospital

Abstract

Introduction: Our aim was to document the range of collagen vascular disease (CVD)-ILD seen at our department and the impact on survival. Results: Of 620 patients with ILD, 144 were found to have CVD-ILD after multidisciplinary team review (27 male, 117 female). At diagnosis, the mean age was 59 years. The CVDs were: progressive systemic sclerosis (PSS); 41, Sjogren9s syndrome (SS); 35, rheumatoid arthritis (RA); 28, undifferentiated-CVD; 14, systemic lupus erythematosus (SLE); 6, vasculitis; 8, anti-Jo-1 syndrome; 4, polymyositis 3, mixed connective tissue disease; 2, ankylosing spondylitis; 2, and antiphospholipid syndrome; 1. The thoracic HRCT available for 136 patients was compatible with non-specific interstitial pneumonia (NSIP); 52 cases, probable idiopathic pulmonary fibrosis (IPF); 27, definite IPF; 15, lymphocytic interstitial pneumonia; 2, early interstitial changes; 12, other patterns; 28. By right heart catheterisation, 18 had pulmonary hypertension (PSS; 11, SLE; 3, RA; 2, undifferentiated-CVD; 2), of whom 12 had early and 6 severe interstitial lung disease. At presentation, the PFTs showed FVC mean 2.19 L FEV 1 /FVC mean 83%, DL CO %predicted mean 58. On Kaplan-Meier analysis, mean survival was 82 months. By comparison with IPF, the survival for CVD-ILD is significantly better (p=0.047). Conclusion: The most common types of CVD-ILD are PSS, SS and RA. The most common pattern is NSIP. Pulmonary hypertension is most common in PSS.