Forced vital capacity as a survival predictor in systemic sclerosis-associated pulmonary hypertension

Background: Pulmonary hypertension associated with systemic sclerosis (SSc-PH) carries a significant risk of mortality. Although interstitial lung disease (ILD) often coexists with SSc-PH, its impact on prognosis varies considerably depending on disease extension. The prognostic implications of different degrees of functional impairment secondary to ILD in patients with SSc-PH remain poorly understood. Objectives: This study aimed to stratify patients with SSc-PH according to their functional parameters and to identify potential prognostic factors in different patterns of ILD extension. Design: A retrospective study was conducted on consecutive patients with SSc-PH diagnosed according to the European Society of Cardiology/European Respiratory Society guidelines for PH since 2015, from two Italian Scleroderma Centers. Methods: Patients were classified according to high-resolution computed tomography (HRCT) findings and forced vital capacity (FVC) values as pulmonary arterial hypertension (PAH) without ILD, PH-ILD with FVC ⩾70%, or FVC