Abstract Rationale Sarcoidosis is a systemic granulomatous disease, often involving the lungs, with a generally good prognosis. However, a subset of patients experiences progressive disease, and cardiac involvement may adversely affect outcomes. Although pulmonary sarcoidosis (PS) is the most common phenotype, data on long-term outcomes and the prognostic impact of coexisting cardiac sarcoidosis (CS) remain limited. Methods We retrospectively analyzed consecutive patients with histologically confirmed PS diagnosed at Hokkaido University Hospital between 2003 and 2024. Baseline assessments included chest X-ray and CT, pulmonary function tests, and echocardiography. When cardiac involvement was suspected based on symptoms or abnormal ECG or echocardiographic findings, 18F-fluorodeoxyglucose positron emission tomography (18F-FDG PET) and cardiac magnetic resonance imaging (CMR) were performed. CS was diagnosed according to the 2016 Japanese Circulation Society (JCS) criteria. Overall survival was estimated using Kaplan-Meier analysis, and prognostic factors were evaluated with Cox proportional hazards models. Results We studied 513 patients with PS, among which 459 were followed for ≥6 months. The mean age was 56 years, and 36% were men. The 3-, 5-, and 10-year survival rates were 98.2%, 97.0%, and 94.6%, respectively (median follow-up 6.7 years). CS was diagnosed in 48 patients (9.4%). In univariate analysis, older age and the presence of CS were significant risk factors for mortality (Figure). After adjustment for age and sex, CS remained an independent predictor of poor prognosis (HR 3.4, 95% CI 1.2-9.4), whereas pulmonary lesion stage and the composite physiologic index (CPI) showed only limited prognostic value(HR 0.96, 95% CI 0.39-2.39 and HR 1.05, 95% CI 1.00-1.09). During follow-up, 21 patients (4.6%) died—mainly from malignancy (48%), cardiac causes (14%), and respiratory failure (14%). Conclusion In this single-center Japanese cohort, overall survival of PS was favorable, yet CS was identified as a strong independent risk factor for mortality. These findings underscore the importance of systematic cardiac screening with modern imaging modalities such as 18F-FDG PET and CMR to enable early detection and risk stratification. Prospective multicenter studies are warranted to validate these results and to further clarify and improve the clinical course of PS. This abstract is funded by: None
Yoshikawa et al. (Fri,) studied this question.