The objective of the current study was to determine survival and factors that affect survival in progressive @entity785 ( @entity785 ) with @entity358 . A total of 102 @entity785 @entity1 with @entity358 , diagnosed between 1994 and 2008, enrolled into the study. @entity358 was defined based on the presence of interstitial changes on @entity2101 ( @entity2101 ). Demographical, clinical, radiological, and laboratory data of the @entity1 were found from @entity1 records and were used for the evaluation of survival. The mean age of the @entity1 at diagnosis was 50      12  years. In follow-up, 22 (21.5%) @entity1 were deceased. The mean age of the @entity1 at death was 55      12  years. Seventy-eight percent of known causes of mortality were related to @entity358 . After any initial systemic manifestation of disease, the mean survival was 447      27  months and 5, 10, 15, and 20  year survival rates were 99, 92, 83, and 72%, respectively. The mean survival after initial pulmonary manifestation was 269      23  months and 5, 10 and 15  year survival rates were 91, 73 and 57%, respectively. Mean survival was 113      5  months, and 5- and 10-year survival rates after the lung involvement detected with @entity2101 were 85 and 66%, respectively. @entity120 , disease onset after the age of 40, and honeycombing on @entity2101 were associated with poor survival. Our data suggest that @entity358 is the most important determinant factor for poor prognosis in @entity1 with @entity785 . Therefore, echocardiography, pulmonary function tests, and @entity2101 should be performed in early stages of the disease for early diagnosis of @entity120 and lung involvement before irreversible vascular and interstitial changes exist.

Survival in progressive @entity785 with XXXX : a single-center experience in Istanbul, Turkey.

@entity1 :: ('9606', 'Species') :: ['patients', 'patient'] @entity120 :: ('MESH:D006976', 'Disease') :: ['Pulmonary artery hypertension', 'pulmonary artery hypertension'] @entity358 :: ('MESH:D008171', 'Disease') :: ['Pulmonary involvement', 'pulmonary complications', 'pulmonary involvement'] @entity2101 :: ('MESH:C538394', 'Disease') :: ['HRCT', 'high-resolution CT'] @entity785 :: ('MESH:D012595', 'Disease') :: ['systemic sclerosis', 'SSc']
@entity358 :: (MESH:D008171,Disease) :: ['pulmonary involvement']
