Physiological predictors of survival in patients with sarcoidosis-associated pulmonary hypertension: results from an international registry

Abstract

IntroductionSarcoidosis-associated pulmonary hypertension (SAPH) is associated with reduced survival in single-centre studies. The international Registry for SAPH (ReSAPH) with long-term follow-up was established to enrich our knowledge of this complication of sarcoidosis. This analysis aims to elucidate factors associated with reduced transplant-free survival in SAPH patients.MethodsReSAPH contains prospectively collected outcomes of SAPH patients since the time of registry enrolment. Information analysed includes right heart catheterisation data, pulmonary function testing, chest radiography, Scadding stage and 6-min walk distance (6MWD), among others. Cox regression models were used to identify independent predictors of transplant-free survival.ResultsData from 215 patients followed for a mean±sd 2.5±1.9 years were available for analysis. In the 159 precapillary patients, the Kaplan–Meier-adjusted 1-, 3- and 5-year transplant-free survival was 89.2%, 71.7% and 62.0%, respectively. Kaplan–Meier-adjusted 1-, 3- and 5-year transplant-free survival in the incident group was 83.5%, 70.3% and 58.3%, respectively, and in the prevalent group was 94.7%, 72.2% and 66.3%, respectively. Patients with reduced diffusing capacity of the lung for carbon monoxide (DLCO) (<35% predicted) and 6MWD <300 m in the precapillary cohort had significantly worse transplant-free survival. Reduced 6MWD and preserved forced expiratory volume (FEV1)/forced vital capacity (FVC) ratio were identified as independent risk factors for reduced transplant-free survival in the precapillary cohort.ConclusionReduced DLCO (<35% pred) and 6MWD (<300 m) at the time of registry enrolment were associated with reduced transplant-free survival in the overall precapillary cohort. Preserved FEV1/FVC ratio was identified as an independent risk factor for worsened outcomes.

Clinical characteristics and outcomes in patients with primary Sjogren's syndrome-associated interstitial lung disease

“…Previous studies in patients with various types of ILD have shown that the parameters obtained from the 6MWT are important physiological markers associated with increased mortality. [ 31 40 41 42 ] In agreement with previous studies, we found that a 6MWD <300 m was significantly associated with reduced survival, highlighting the importance of the 6MWT as a surrogate marker of disease severity in pSS-ILD patients. In univariate Cox regression analysis, a number of factors were associated with the survival of patients with pSS-ILD; however, in multivariate analysis, we found that male sex, UIP pattern, and a reduced percent predicted FVC were the only independent predictors of increased mortality.…”

Long‐term tolerability of phosphodiesterase‐5 inhibitors in pulmonary hypertension of sickle cell disease

“…This is problematic as 60% of patients with PH of SCD have elevated left‐sided filling pressures, which are also associated with increased mortality 17 . This phenomenon is similar to what is observed in another form of Group 5 PH, related to sarcoidosis 21 . We utilized the historical definition of PH (mPAP of ≥25 mm Hg) as part of our study design as this was the definition at the time of PDE5‐I utilization.…”

Sarcoidosis-Associated Pulmonary Hypertension

“…10 The PH prevalence highly depends on the selected population. PH presents more often in patients with stage IV sarcoidosis, 11,12 and the prevalence increases as the disease proceeds over years. 9 In patients with persistent dyspnea, PH prevalence rises up to 38 to 45%.…”

“…Identified risk factors for worse prognosis might be Scadding stage IV sarcoidosis, 13 precapillary PH without a postcapillary component (HR: 10.4 compared with 3.14), 13 right atrial pressure >15 mm Hg, 75 DLCO, 12,17 6MWD < 300 m, 12 and right ventricular failure. 88,101 Results on reduced FVC showed various results.…”

“…88,101 Results on reduced FVC showed various results. 12,13 Conclusion PH is a well-known complication of sarcoidosis, defined by a mean PAP of !25 mm Hg. Since both PH and sarcoidosis are rare diseases, data on SAPH come mostly from small retrospective studies.…”