Abstract

Abstract Systemic sclerosis is a connective tissue disease characterized by endothelial dysfunction, hyper reactivity of fibroblasts and immunity abnormality in both humoral and mediated cells. Clinically, in addition to the typical skin involvement, mainly the face and fingers, is frequent involvement of lungs, heart and kidneys. Cardiac involvement, in combination with lung involvement, is considered to be the most important determinant of the prognosis of these patients. Cardiac involvement occurs in 15– 35% of cases but in autopsy surveys the incidence reaches 80%. The case we present concerns a woman of 65 years, admitted in December at the Department of Cardiology of our AOU for an episode of low–output heart failure with epigastralgia, sweating, and breathlessness. The cardiological history was almost silent; instead, it presented a history of chronic thyroiditis and a recent infection with SARS COV 2. The objective examination presented hypokinesis of basal pulmonary fields and pitting edema. The blood tests showed an increase in myocardial necrosis, BNP, creatininemia and inflammatory indices (VES, PCR, beta 2 microglobulin, ferritin, fibrinogen), normocytic normochromic anemia, hypogammaglobulinemia, hyperuricemia and proteinuria and hematuria. The echocardiogram showed severe biventricular dysfunction and moderate–grade mitral insufficiency. A coronary examination was performed that showed epicardial coronary vessels free from significant injury. The cardiac magnetic resonance showed a widespread late enhancement subendocardial of the ventricular and atrial chambers with non–vascular distribution suggestive of pathology on an autoimmune basis. Upon closer examination, multiple small telangiectasias were observed in the face, slight hyperpigmentation of the chest and back, and swelling of the hands. The antibody panel showed positivity of ANA and anti Scl70. The patient was thus addressed to a multi–specialist management in which therefore cardiologist and rheumatologist compare and support the treatment of this pathology with the goal of reducing the high mortality and morbidity that characterizes systemic scleroderma with cardiac involvement.

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