PO81 - FVC Decline in Advanced Pulmonary Sarcoidosis is Associated with Reduced Survival: Results of the ReAS Study
Ashley Cattran (United States)1; Ogugua N. Obi (United States)2; Robert P. Baughman (United States)1; Marc Judson (United States)3; Surinder Birring (UK)4; Helmi Van Hirtum (Netherlands)5; Marcel Veltkamp (Netherlands)5; W. Ennis James (United States)6; Lisa Maier (United States)7; Violetta Vucinic (Serbia)8; Eva Carmona (United States)9; Mehdi Mirsaedi (United States)10; Vasileios Kouranos (UK)11; Athol U. Wells (UK)11; Elyse E. Lower (United States)1;
1 - University of Cincinnati; 2 - East Carolina University; 3 - Albany Medical Center; 4 - King's College Hospital; 5 - Sint Antonius Hospital; 6 - Medical University of South Carolina; 7 - National Jewish Health; 8 - University of Belgrade; 9 - Mayo Clinic; 10 - University of Florida; 11 - Royal Brompton Hospital;
Keywords: Advanced sarcoidosis; FVC decline; Mortality;
Select the theme: Challenges in Sarcoidosis Diagnosis and Staging
Type: Original Papers
Presentation: Poster Presentation

Introduction: Patients with advanced pulmonary sarcoidosis have significant mortality. Identifying predictors of mortality is essential to guide timely management. In other interstitial lung diseases, a fall in forced vital capacity (FVC) over 12 months has been associated with increased mortality.  

Objectives: We analyzed a large cohort of advanced pulmonary sarcoidosis patients from the Registry for Advanced Sarcoidosis (ReAS) to determine if a decline in FVC at one-year was associated with decreased survival.

Materials and Methods: Entry criteria for enrollment in ReAS included sarcoidosis patients with advanced pulmonary disease (significantly impaired pulmonary function, fibrotic lung disease, or pre-capillary pulmonary hypertension), cardiac disease, neurologic disease or receiving third line therapy. A contemporaneous control population was enrolled and matched for age, race, and gender. Data including spirometry and Kings’ Sarcoidosis Questionnaire-Lung (KSQ-L) were collected at baseline and at regular intervals. Clinical status was determined as of 12/31/2025 or at the last known contact date. Kaplan-Meier survival analysis was performed, and results are reported as hazard ratios (HRs)for mortality with 95% confidence intervals (CIs), and p-values <0.05 considered statistically significant.

Results: A subset of 86 patients with initial and 12-month follow-up data was analyzed. Overall, 21% of patients died or were transplanted during a median follow-up of 7.2 years. An absolute decrease in FVC % predicted of 5% or greater was associated with increased mortality (HR 3.1 (CI: 1.15 to 8.10, p=0.024). Additionally, FVC decline > 150 ml was associated with a 3.8-fold increased mortality (HR = 3.84 (CI: 1.46 to 10.06, p=0.006). Eighteen patients experienced a decrease in KSQ-L of 4 or more points without associated increase in mortality (HR =1.19 [CI: 0.373 to 3.795, p=0.086).

Conclusions: A significant decrease in FVC (>5% predicted or 150 mL) at 12-months was associated with reduced survival in patients with advanced pulmonary sarcoidosis.

Change in survival with FVC decline 5% or more at 12 month follow upChange in survival with FVC decline 150 ml or more at 12 month follow up