Long-term risk of mortality and XXXX and predictive classification of primary Sj  @entity244 .

@entity1 @entity1071 @entity706 @entity1283 @entity4125 @entity244 @entity204
OBJECTIVE: Primary Sj  @entity244 ( @entity244 ) may lead to @entity1071 ( @entity1071 ) and @entity204 in certain @entity1 . We sought to determine the incidence and predictors of adverse long-term outcomes to achieve a rational predictive classification of the syndrome. METHODS: Predictive modeling was performed in a cohort of 723 consecutive @entity1 with primary @entity244 (587 newly diagnosed [incident] cases and 136 prevalent cases). RESULTS: During 4,384 @entity1 -years of followup, we recorded 39 deaths (7 due to @entity706 ) and 38 diagnoses of @entity1071 . The standardized mortality ratio was 1.15 (95% confidence interval [95% CI] 0.86-1.73) compared with the general population of Greece. In incident cases, the probability of @entity1071 was 2.6% at 5 years and 3.9% at 10 years. Mortality rates were significantly higher in @entity1 with low C4 levels at the first study visit (hazard ratio [HR] 4.39, 95% CI 2.18-8.83). @entity1071 was independently predicted by the presence of @entity1283 (HR 5.21, 95% CI 1.76-15.4), palpable @entity4125 (HR 4.16, 95% CI 1.65-10.5), and low C4 levels (HR 2.40, 95% CI 0.99-5.83) at the first study visit. All @entity1 who eventually developed @entity706 resulting in @entity204 during the followup period had either low C4 levels or palpable @entity4125 at the first study visit. Training-validation split-cohort modeling confirmed the predictive importance of low C4 levels and palpable @entity4125 , both of which were present in 20.9% of @entity1 at their first visit. CONCLUSIONS: In @entity1 with primary @entity244 , 1 in 5 deaths is attributable to @entity706 . The presence of palpable @entity4125 and low C4 levels at the first visit adequately distinguishes high-risk @entity1 (type I primary @entity244 ) from @entity1 with an uncomplicated disease course (type II [low-risk] primary @entity244 ).
