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

Marc Schmalzing; Pia Moinzadeh; Jithmi Weliwitage; Joerg Henes; Norbert Blank; Gernot Keyßer; G Riemekasten; Ulf Müeller-Ladner; M Worm; Aaron Juche; Alexander Kreuter; Jan Ehrchen; Laura Susok; Claudia Guenther; G. Zeidler; Ilona Jandova; Tim Schmeiser; Christiane Pfeiffer; Ina Koetter; A. Alberding; Joerg H W Distler; Nicolas Hunzelmann

doi:10.1093/rheumatology/keag155

Key Points

To investigate the effects of pulmonary hypertension treatment on survival outcomes in systemic sclerosis patients with and without interstitial lung disease.
Analyzed a large cohort of systemic sclerosis patients.
Evaluated treatment strategies involving pulmonary hypertension combination therapy.
Assessed the severity of interstitial lung disease in patients.
Examined survival outcomes associated with different treatment approaches.
Combination therapy for pulmonary hypertension is feasible for SSc patients.
Treatment does not worsen survival in pulmonary hypertension patients with mild interstitial lung disease.
Severity of interstitial lung disease influences treatment outcomes.

Implication

Large cohort study shows treatment impacts survival in patients with systemic sclerosis and pulmonary hypertension, highlighting clinical significance.

Abstract

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.

Discussion

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Cite This Study

Schmalzing et al. (2026) studied this question.

synapsesocial.com/papers/69e31ff140886becb653f13c https://doi.org/10.1093/rheumatology/keag155

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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

Key Points

Implication

Abstract

Discussion

Cite This Study

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

Key Points

Implication

Abstract

Discussion

Cite This Study

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