Treatment of systemic sclerosis-associated pulmonary hypertension with and without interstitial lung disease and its impact on survival: a large cohort study of the German network for systemic sclerosis.

Abstract

This study aimed to analyse survival in SS-associated pulmonary hypertension (SSc-PH) patients with or without interstitial lung disease (ILD) stratified by PH monotherapy and PH combination therapy, respectively. Among the 6003 patients registered with the German Network for Systemic Sclerosis (DNSS) registry, SSc patients with incident PH diagnosed after registry entry were included. Kaplan-Meier analysis was performed to estimate the overall survival, and the log-rank test was used to assess differences between groups stratified by the presence of ILD or PH treatment. Of, the 384 patients who fulfilled the inclusion criteria, 271 had concomitant ILD (70.6%). The mean follow-up period was 8.69 ± 4.94 years. Forced vital capacity (FVC) and diffusing capacity of the lungs for carbon monoxide (DLCO) numerically decreased in PH patients with and without ILD and were significantly lower in patients with ILD (mean value of minimal FVC during follow-up: 70.79 ± 24.36%). There was no significant difference in the distribution of PH therapy categories (mono, dual, or triple therapy) between PH patients with and without ILD involvement (P = 0.865). No survival difference was observed between the monotherapy and combination therapy groups (P = 0.197). Subgroup analysis indicated that varying the treatment strategies did not significantly impact the survival outcomes of PH patients, whether or not ILD was present. SSc patients with ILD and incident PH are often treated with PH combination therapy in Germany. This treatment strategy seems feasible and does not worsen survival in PH patients, provided that ILD is not severe.