Impact of systemic sclerosis-associated interstitial lung disease with and without pulmonary hypertension on survival - a large cohort study of the German network for systemic sclerosis

BACKGROUND: Pulmonary involvement is the leading cause of death in systemic sclerosis (SSc) and may manifest as interstitial lung disease (ILD), pulmonary arterial hypertension (PAH), or in combination of both (ILD-pulmonary hypertension (PH)). Aim of this analysis was to determine prevalence, clinical characteristics, and survival of these different forms within the registry of the German Network for Systemic Sclerosis …