RESUMO
Systemic sclerosis (SSc) is a chronic autoimmune disease with complex pathogenesis, characterized by vascular dysfunction and fibrosis. Digital ulcers (DUs) are a common and severe complication in SSc patients, negatively impacting their quality of life. This retrospective study evaluates the use of macitentan, an endothelin receptor antagonist, in six female patients with connective tissue disease (CTD) and sclerodermiform features (five SSc and one mixed connective tissue disease) for the treatment of refractory DUs. Macitentan demonstrated a safe and effective alternative to bosentan, reducing DU relapses, hospitalizations, and the use of systemic prostaglandin therapy. The findings suggest that macitentan may be a valuable therapeutic option in specific cases of recurrent or refractory DUs and warrant further investigation in larger, long-term studies.
RESUMO
BACKGROUND: Cardiac involvement is one of the most frequent manifestations of Systemic Lupus Erythematosus (SLE). Transthoracic echocardiogram (TTE) may be valuable for the early detection of cardiac abnormalities in SLE. Few studies analyze both TTE findings in SLE and the risk factors that predispose to different cardiac manifestations in a long follow-up cohort. We aimed to investigate cardiac involvement's prevalence, risk factors, and outcomes in a Spanish Lupus Clinic. METHODS: Spanish single-center prospective study of cardiac involvement in SLE. Two hundred and one patients met the 2019 EULAR/ACR classification criteria, performed TTE, and were eligible for the study. RESULTS: Cardiac involvement was present in 43.8%. Patients with older age, hypertension, hyperlipidemia, higher body mass index, peripheral arterial disease, thrombosis, and major cardiovascular events had significantly more cardiac involvement. Neurological, hematological, and serosal involvement (pleuritis and/or pericarditis) were clinical risk factors for abnormal TTE. The combination of the four clinical variables (dyspnea, chest pain, cough, and/or syncope) was present in 40.9% of the patients with abnormal TTE in the follow-up and was superior to each of the manifestations separately. Troponin I (TnI) ≥ 0.2 ng/mL and NTproBNP ≥ 300 pg/mL were excellent biomarkers with a good correlation with cardiac abnormalities. Anti-B2GP1 was the only autoantibody associated with cardiac involvement in our cohort. Presenting cardiac involvement was correlated with higher SLICC Damage Index and increased mortality risk in the 2-year follow-up period. CONCLUSIONS: Cardiac involvement in SLE is diverse, heterogeneous, and highly prevalent. Presenting a pathological TTE was associated with greater damage accrual and greater mortality. Based on our results, we consider that echocardiographic screening of patients with SLE is essential, especially those symptomatic and/or with risk factors, to diagnose and treat cardiac involvement earlier.
