15 February 2020 by Ricardo Gomez Tejada

Screening of pulmonary hypertension in scleroderma and idiopatic inflammatory myopathies patients

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.

About the author


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Ricardo Gomez Tejada

ALAT PULMONARY CIRCULATION DPT. DIRECTOR

HOSPITAL DE CLÍNICAS. UNIVERSIDAD DE BUENOS AIRES

Argentina

Key Contributors

Gómez Tejada RA, Laborde H, Gómez R, Braillard Poccard A, Mastandrea N, Giovini V, Monteverde M, Barrios V, Mazzei M, Vázquez Blanco M Hospital de Clínicas “José de San Martín”. Facultad de Medicina, Universidad de Buenos Aires. Argentina.


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