Myocarditis is a rare but life-threatening complication of systemic sclerosis (SSc). Here we present the case of a 38-year-old man who presented with rapidly progressive diffuse cutaneous systemic sclerosis (dcSS) with skin, gastrointestinal, lung, and cardiac involvement in the form of myocarditis. His myocarditis has been successfully controlled with pulsed intravenous (IV) cyclophosphamide and methylprednisolone. Serial cardiac magnetic resonance imaging (MRI) scans have demonstrated disease stability in the interim.The 38-year-old male, a landscape gardener, presented with a 6-month history of arthritis affecting the small joints of his hand, Raynaud's disease, and sclerodactyly. Blood pressure and urinanalysis were normal. Antinuclear antibody (ANA) and anti-Scl-70 antibody tests were positive. Rheumatoid screen was negative. The clinical impression was that of early dcSS and the patient was commenced on a calcium channel blocker and prednisolone 20 mg once daily.The patient's disease progressed rapidly, with skin tightness developing over the face, chest wall, back, forearms, and hands. Suspected oesophageal dysmotility was confirmed by oesophageal manometry. Renal indices remained normal.In September 2008 the patient developed exertional dyspnoea. Pulmonary function tests, echocardiogram, and right heart catheterization were normal. High-resolution computed tomography (CT) of the thorax showed no evidence of pulmonary fibrosis but did reveal extensive mediastinal lymphadenopathy. Lymph node and lung biopsy were obtained by video-assisted thorascopic surgery (VATS). Histology revealed reactive lymphadenopathy and desquamative interstitial pneumonitis indicating early pulmonary involvement of SSc.Shortly after the VATS procedure the patient developed chest pain at rest, elevated cardiac enzymes (creatine kinase 494 U/L), troponin leakage (troponin T 0.39 μg/L), and intermittent atrio-ventricular re-entrant tachycardia. Coronary angiogram was normal. A cardiac MRI was obtained that demonstrated normal ventricular function and no obvious abnormal subendocardial enhancement (Figure 1).Although symptoms initially settled they recurred over the following 6 weeks with further chest pain at rest, recurrent dysrhythmias, persistently raised cardiac enzymes, and troponin leakage that proved refractory to treatment with oral steroids and anti-arrhythmic agents. A repeat cardiac MRI 6 weeks after the previous MRI showed a deterioration of ventricular function with a right ventricular ejection fraction of 25% and a left ventricular ejection fraction of 39%. In addition, abnormal subendocardial enhancement was noted in the left ventricular papillary muscle and the right ventricular trabeculum septum marginale (Figure 1). The MRI findings confirmed the clinical impression of an acute myocarditis secondary to SSc and the patient was commenced on pulsed IV cyclophosphamide (10 mg/kg monthly).Unfortunately, cardiac enzymes remained persistently elevated 3 days following the start of treatment indicating ongoing myocarditis. ...
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