Survival and XXXX risk in an unselected and complete Norwegian @entity5442 cohort.

@entity1 @entity43 @entity6017 @entity5442 @entity5 @entity358 @entity5589 @entity204 @entity6558
OBJECTIVE: To utilise an exposed/unexposed cohort strategy for mortality and @entity5 analyses across unselected and complete cohorts of @entity1 with @entity5442 ( @entity5442 ) resident in south-east Norway (denominator population 2.6 million), between 2003 and 2012. METHOD: @entity5442 cases were identified by comprehensive searches through @entity1 administrative databases followed by manual chart review. @entity6558 ( @entity6558 ) and @entity6017 ( @entity6017 ) cases were classified by the Peter and Bohan and/or Targoff diagnostic criteria and @entity5589 ( @entity5589 ) by the European NeuroMuscular Centre (ENMC) criteria from 1997 and/or 2011. Every @entity1 was matched for sex, age and residential area with 15 unexposed/non- @entity5442 individuals drawn from the national population registry. RESULTS: Total mortality in the @entity5442 cohort was 27% (87/326). Standardized mortality rate (SMR) was higher in @entity6017 (2.6) than @entity6558 (2.4) and @entity5589 (1.7). @entity5442 -related causes of @entity204 were frequent (64%) and included @entity5 (all @entity5442 subsets), aspiration ( @entity5589 ), @entity358 ( @entity6558 / @entity6017 ) and infections ( @entity6558 / @entity6017 ). Multivariate analyses identified age at diagnosis ( @entity6558 and @entity5589 ), positive anti-SSA ( @entity6558 ), @entity5 ( @entity6017 ), and DLCO < 60% ( @entity6017 ) as independent mortality risk factors. @entity5 risk was increased in @entity6017 (standard incidence rate 2.0) and @entity6558 (SIR = 1.3), but not in @entity5589 (SIR = 0.9). @entity43 was more prevalent in @entity6017 than in the general population (8.3% vs 1.1%). CONCLUSION: Our results suggest that mortality rates and @entity5 risk remain elevated in @entity6017 , and to a lesser degree also in @entity6558 . Mortality rate was also increased in @entity5589 , but some @entity204 appeared to be due to potentially preventable causes.
