Title: Scleroderma Associated Autoimmune Myocarditis Authors: Hanyuan Shi, MD. Asaad Nakhle, MD. Introduction:
Systemic sclerosis (SSc) affects the cardiovascular system in a myriad of different manners. Some of these processes can be chronic, while others can be acute such as myocarditis. This is a challenging case of a patient presenting with acute atrial flutter and heart failure, who was found to have systemic sclerosis with autoimmune myocarditis. Cardiac magnetic resonance (CMR) imaging was used to both diagnose the primary cardiac involvement in scleroderma as well as highlight the extent of the disease process. Case Report: A 72-year-old woman with HTN, PVD, moderate MR, presented with one month of bilateral discoloration of her fingers and weight loss. Exam showed gross volume overload, gangrene to her 4 th digits, a “salt and pepper” rash of her scalp, sclerodactyly, and a narrow oral aperture. Labs showed hypoalbuminemia, positive ANA, and positive Anti-Scl70 reactivity. EKG showed atrial flutter with RVR, and TTE showed LVEF of 36- 40%, impaired RV systolic function, severe MR, and moderate MS. Tc-99m scan showed no evidence of transthyretin (TTR) cardiac amyloidosis. Cardiac MRI confirmed the TTE with additional late gadolinium enhancement (LGE) findings of multifocal linear mid-myocardial and epicardial enhancement suggestive of an autoimmune myocarditis process. These lab and imaging findings were consistent with scleroderma, Raynaud’s with digital ulcers, and myocarditis. Discussion: Cardiac involvement in SSc can occur 4-5 years after onset of initial disease. These manifestations can occur as heart failure, pericarditis, autonomic dysfunction, but very rarely as acute autoimmune myocarditis. This is a form of primary cardiac involvement, that usually involves inflammatory infiltrates into the heart. CMR with LGE confirmed multifocal enhancement and focal myocardial edema, leading to the diagnosis. Conclusions: The optimal therapy for autoimmune myocarditis in the setting of biventricular heart failure and scleroderma is unknown. Although patients can be treated with immunosuppressants and diuretics, late stage fibrosis cases can have high mortality rates.
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