The aim of our study was to study the changes in the clinical picture and outcomes of @entity2883 with @entity2883 (Wegener's) (GPA) over 40  years. Two hundred and forty-two consecutive @entity1 with GPA were distributed into retrospective (1970-2003) and prospective (2004-2012) cohorts. Anti-neutrophil cytoplasmic antibodies were present in 82.6  % of @entity1 . In 78.0  % of @entity1 , diagnosis was confirmed histologically. We compared the clinical features of GPA and the incidence of the major and minor relapses and mortality in the two cohorts. The majority of @entity1 in both cohorts had generalized GPA that involved upper respiratory tract (retrospective 89.5  % vs prospective 82.85  %), kidneys (60.5 vs 50.8  %) and lungs (64.0 vs 52.3  %). The total duration of follow-up in the retrospective and prospective cohorts was 468 and 397 @entity1 -years, respectively. In the prospective cohort, we found trend to lower incidence of relapses (54.2 vs 66.2 per 100 @entity1 -year; p  =  ns; odds ratio 0.82; 95  % CI 0.53-1.21) and significantly lower mortality (4.3 vs 7.9 per 100 @entity1 -year; p  =  0.04; odds ratio 0.54; 95  % CI 0.31-0.94). The leading causes of @entity204 in the retrospective cohort were @entity358 (37.8  %), complications of immunosuppressive treatment (35.1  %) and @entity177 (13.5  %). In the prospective cohort, @entity1 rarely died from terminal uraemia and @entity358 (0.0 and 17.6  %) while the proportion of cardiovascular events and complications of the immunosuppression as the causes of @entity204 increased (29.4 and 47.1  %). Modern treatment apparently reduced the incidence of relapses and mortality and modified the causes of @entity204 in the GPA @entity1 .

Changing patterns of clinical severity and risk of mortality in @entity2883 with XXXX over four decades: the Russian experience.

@entity1 :: ('9606', 'Species') :: ['patients', 'patient'] @entity177 :: ('MESH:D051437', 'Disease') :: ['kidney failure'] @entity358 :: ('MESH:D008171', 'Disease') :: ['lung disease', 'pulmonary complications'] @entity2883 :: ('MESH:D014890', 'Disease') :: ['polyangiitis', 'granulomatosis'] @entity204 :: ('MESH:D003643', 'Disease') :: ['death']
@entity2883 :: (MESH:D014890,Disease) :: ['polyangiitis']
