Successful control of scleroderma myocarditis using a combination of cyclophosphamide and methylprednisolone

Abstract

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. ...

Silent myocarditis in systemic sclerosis detected by cardiovascular magnetic resonance using Lake Louise criteria

“…Although myocardial involvement in SSc was usually attributable to non-inflammatory causes, there are early reports presenting SSc patients with evidence of myocarditis and good response to the combination of either corticosteroids and azathioprine [11] or corticosteroids and cyclophosphamide [12]. Our results are in agreement with the majority of previous studies detecting myocarditis in SSc [7–9, 13–15, 17, 18].…”

Fatal Myocarditis in a Male with Systemic Sclerosis

“…Patient is given cyclophosphamide pulses (10 mg/kg monthly) and intravenous methylprednisolone with a total of nine doses of cyclophosphamide and three courses of methylprednisolone. Good clinical and biological evolution is noted [6]. Allanore treat the same way, scleromyositis overlap syndrome and polymyositis related myocarditis with intravenous cyclophosphamide with a good outcome [17].…”

“…Myocarditis is a rare clinical condition in patients with SS, only a few cases are reported [5,6]. In a cohort of 181 patients with SS, seven patients develop acute clinical symptoms of heart disease, among them six have an acute myocarditis (3.3%) [7].…”

“…But all authors agree that corticosteroids are mandatory to treat muscular inflammation. High dose steroid (1 mg/kg/day) is often needed in SS like in all inflammatory myopathies [6,17], it may be or not started with intravenous methylprednisolone. In these patients, high risk of scleroderma renal crisis decreases if angiotensin converting enzyme inhibitors are given.…”

Arrhythmogenic Inflammatory Cardiomyopathy in Autoimmune Rheumatic Diseases: A Challenge for Cardio-Rheumatology

“…In SSc patients who develop myocarditis/AIC, timely initiation of immunosuppressive treatment, such as cyclophosphamide and corticosteroids [76], in parallel with adequate cardiac medication, can delay the VT development and the progression of cardiac dysfunction. It is possible that a subclinical but impaired myocardial performance can be improved by moderate corticosteroid doses in the short-term [77], but this should be further confirmed by double-blind controlled studies.…”