PT  - JOURNAL ARTICLE
AU  - Nesrin Mogulkoc
AU  - Imren Nesil
AU  - Aysu Sinem Koc
AU  - Zehra Erdemir
AU  - Selen Bayraktaroglu
AU  - Yasemin Kabasakal
AU  - Canan Gunduz
AU  - Pervin Korkmaz Ekren
AU  - Feza Bacakoglu
TI  - Survival in CVD-ILD at Ege University Hospital
AID  - 10.1183/13993003.congress-2016.PA4880
DP  - 2016 Sep 01
TA  - European Respiratory Journal
PG  - PA4880
VI  - 48
IP  - suppl 60
4099  - http://erj.ersjournals.com/content/48/suppl_60/PA4880.short
4100  - http://erj.ersjournals.com/content/48/suppl_60/PA4880.full
SO  - Eur Respir J2016 Sep 01; 48
AB  - 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, Sjögren's 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 FEV1/FVC mean 83%, DLCO %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.