PROGNOSTIC FACTORS FOR SEVERE PULMONARY INVOLVEMENT IN SYSTEMIC SCLEROSIS

Background. Lung involvement is the main disease related death cause in patients with systemic sclerosis (SSc). The most frequent lung manifestations in SSc are interstitial lung disease (ILD) and pulmonary arterial hypertension (PAH). Objectives. Evaluation of lung involvement in patients with SSc and identification of predictive factors for severe lung involvement. Patients and methods. All patients with SSc of the EUSTAR100 center, having at least two visits between 2004 and 2016, were included. Survival status, cause of death, dyspnea, ILD on thorax radiography or high resonance thorax computer tomography (HRCT) and lung function tests were recorded during the entire follow-up. Severe lung involvement was defined as severe or end-stage lung involvement on the Medsger severity scale at any time during follow-up, or death. Cox proportional hazards regression was used in univariate and multivariate analysis to identify prognostic factors. Results. 89 patients were included (12.4% males, mean age±SD 49.2±12.2 years, disease duration 4.1±7.5 years), with a follow-up duration up to 13 years. 14 deaths were reported, half due to lung involvement (4 deaths due to ILD, 3 deaths due to