Systemic sclerosis & Raynaud's
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
Authors' conclusion
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.
Study context
- Population
- SSc patients with incident PH diagnosed after entry into the DNSS registry (6003 registered patients), with or without interstitial lung disease (ILD)271 with ILD (70.6%)
- Participants
- 384 patients
- Exposure
- PH monotherapy vs PH combination therapy, stratified by presence of ILD
- Outcome
- Overall survival
Methodological detailsGerman Network for Systemic Sclerosis (DNSS) registry, Kaplan-Meier analysis, log-rank test
- German Network for Systemic Sclerosis (DNSS) registry
- Kaplan-Meier analysis
- Log-rank test
Main finding
As reported in the abstract
- Combination therapy vs Monotherapy: P = 0.197
- Mean follow-up 8.69 ± 4.94 years
Observational data: associations, not effects.
Further findings
| Outcome | Values |
|---|---|
| Minimal FVC during follow-up, patients with ILD | ILD mean 70.79 (SD 24.36) % |
Survival by treatment strategy, with and without ILD (subgroups)
- Varying treatment strategies did not significantly impact survival of PH patients, whether or not ILD was present
Distribution of PH therapy categories (mono, dual, triple), with vs without ILD
- No significant difference
- P = 0.865
Minimal forced vital capacity (FVC) during follow-up, patients with ILD
- FVC and DLCO numerically decreased with and without ILD and were significantly lower with ILD
Original publication
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. Rheumatology (Oxford). Published 15 April 2026. doi:10.
Composed by AI from the published abstract and checked against it. How Briefs are made →