Primary heart disease in systemic sclerosis (scleroderma): advances in clinical and pathologic features, pathogenesis, and new therapeutic approaches.

摘要: Heart disease in SSCL may be primary or secondary. Primary involvement includes pericarditis, myocardial fibrosis, and contraction band necrosis with congestive cardiomyopathy, conduction system intramural coronary artery lesions and, rarely, valvular disease. Symptoms include those of left right ventricular failure, chest pain, palpitations, syncope, sudden death. Pathogenesis related to functional Raynaud's phenomenon the heart and/or structural small vessel Therapy at present is symptomatic; however, new therapeutic approaches are warranted.