Background Pulmonary arterial hypertension (PAH) is one of the leading causes of death in Systemic Sclerosis (SSc). World Health Organization (WHO) functional class at diagnosis is one of the most reliable outcome predictors of PAH. Moreover, patients starting in a poor functional class (eg. class III-IV) have a favorable long-term course if they achieve a better class (eg. class II) after initiation of PAH-targeted therapy. Therefore, current guidelines suggest a goal-oriented treatment aimed at obtaining a good functional class.
Objectives To evaluate the proportions of patients with SSc-associated PAH in WHO functional class II at time of diagnosis and during follow-up.
Methods In this retrospective single-center study all patients affected by SSc-associated pre-capillary PAH diagnosed between 2001 and 2014 were included, excluding those with clinically significant interstitial lung disease (ILD; Forced Vital Capacity <70%), a SSc-related complication affecting functional class.
Results PAH was diagnosed in 41 patients with SSc. Six of them (15%) were excluded because of associated ILD. Among 35 patients included, 11 (35%) were in WHO functional class II at the time of diagnosis, and 24 in class III-IV. Other main demographical and clinical characteristics of these patients were: limited cutaneous SSc: 35 (100%); female: 34 (97%); median age (IQR) 71.5 (64-75) years; time since Raynaud's phenomenon (RP) onset: 21 (13-31) years; time since first non-RP symptom: 10 (2-15) years; ANA+ 34 (97%); anticentromere+: 27 (77%); anti-Topo I+: 0; anti-RNA polymerase III+: 1 (3%); sPAP estimated by echocardiography: 74 mmHg (58-88); TAPSE: 14 mm (12-19); FVC 115% (104-130); DLCO: 40% (36-52); 6 minutes walking test distance: 242 m (168-311); mean PAP on right heart catheterization: 38 mmHg (33-49); Cardiac Index: 2.9 l/min/m2 (2.1-3.5); PVR: 5.4 UW (3.5-6.4).
Patients were divided in two groups according the time of diagnosis: 13 were diagnosed after 2010 and 22 before 2010. In patients with more recent diagnosis WHO functional class II was more frequent than in the others (7/13 (53%) vs. 4/22 (18%); p:0.03). There were trends toward better haemodynamic parameters in the first group, but these did not reach statistical significance.
Twenty-one patients died; median survival from diagnosis of PAH was 49.5 months. Survival was better in patients diagnosed after 2010, as compared with those diagnosed before (log-rank test: p: 0.048; figure 1).
At January, 2015, 14 patients were alive: in 8 of them the diagnosis was done in WHO functional class II, and all were still in this functional class; in 6 patients the diagnosis was done in WHO class III, and 3 of them were in WHO class II at the last evaluation. Therefore, the majority of patients in follow-up (11/14; 76%) had a good functional class at the moment of the last evaluation.
Conclusions This retrospective evaluation of a real-life setting shows that diagnosis of SSc- associated PAH in a good functional class was rare before 2010, but it became more frequent in the last years. Moreover, the majority of patients can obtain a good functional class with PAH-targeted therapy. These data reflects better awareness of the problem and better strategies of diagnosis and of treatment, and may be linked to a better survival.
Disclosure of Interest None declared
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