Rationale: Pulmonary Hypertension (PH) prevalence in Connective Tissue Diseases (CTD) highlights the value of regular screening. Clinical, serologic and comorbilities profils are described in a series of iSSc and IIM patients..
Methods: Retrospective study from an institutional outpatient sample during years 2008 to 2018. Demographic and clinical features were evaluated. Pulmonary Function tests (PFTs), CT Scans and Inmunoserology tests were performed. . We defined comorbilities as Interstitial lung disease (ILD), chronic tromboembolic disease (CTED) and left heart disease (LHD) .Inclusion criteria were: patients older 16 y.o.; ACR/EULAR ’13 SSC criteria and EULAR/ ACR’17 criteria for IIM. PH was defined with these TransThoracic Echocardiography (TTE) features: Doppler Pulmonary Artery Systolic Pressure (PASP) ≥ 40 mm Hg; ; right ventricular enlargement (VD/VI > 1); interventricular septum flattening
Results: We evaluated 217 patients with CTD: 129 (SSc) and 88 (IIM). TTE features for PH were fulfilled in 17 (13,2%) SSc patients and 5 (5,7%) IIM patients. All of them showed low tolerance to exercise and frequent dyspnea. WHO functional class was ≥ III in all. Five patients received Right Heart Catheterization (RHC) with médium Pulmonary Arteial Pressure > 45 mmHg . Results in Table.
Conclusions: 1) the screening of PH in SSc is mandatory. A similar approach in IMM should be reevaluated. 2) anticentromere antibodies (ACA) and U1RNP antibodies were indicative of PH in this CTD series; 3) SSc showed association with ILD, expressing PH Group III; 4) Frequent comorbilities in SSC are compatible with multiple pathophysiologic mechanisms of PH.; 5) superposition conditions in CTD were frequent.