Abstract

Background: Sarcoidosis is a multi-organ granulomatous disease with a variable course. To predict mortality in routine clinical practice is challenging, thus, it is imperative to identify predictors of mortality in pulmonary sarcoidosis to guide therapy and management effectively. This study aims to evaluate predictors of all-cause mortality at 5 years in patients with pulmonary sarcoidosis from a Spanish cohort. Study design: We developed a retrospective, multicentre analysis from the Spanish pulmonary sarcoidosis cohort (SARCO-I), which included 765 patients diagnosed according to 1999 WASOG criteria. Demographic, clinical, radiological, functional, and laboratory characteristics were analysed. Composite physiological index (CPI) at diagnosis and functional and imaging progression according to the progressive pulmonary fibrosis (PPF) criteria, were also assessed. Univariate and multivariate analyses were performed using Cox proportional hazards models to evaluate predictors of 5-year all-cause mortality. Results: At 5 years from diagnosis, 43 (5.6 %) deaths were reported. Upon multivariate analysis, age at diagnosis and baseline CPI emerged as independent predictors of mortality at 5 years. The age threshold of over 50 years (HR 21.16, CI 95 % 2.75-162) and a CPI >35 (HR 2.84, CI 95 % 1.06-7.6) demonstrated the best discrimination of mortality. The presence of fibrosis at diagnosis, functional deterioration, and radiological progression within the first year did not emerge as predictors of mortality. Conclusion: Age over 50 years and a CPI greater than 35 were identified as independent predictors of 5-year allcause mortality in our Spanish pulmonary sarcoidosis cohort.