Long-term risk of mortality and lymphoproliferative disease and predictive classification of primary Sjögren's syndrome.

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