Endocardial and myocardial involvement in systemic sclerosis – is there a relevant inflammatory component?

ObjectivesMyocardial manifestations of systemic sclerosis are mainly due to fibrotic remodeling. We report on two cases, where an endocardial and myocardial inflammation may be a relevant component of cardiac disease.Case seriesCase 1 presented with fulminant tricuspid failure in the absence of pulmonary hypertension and with newly developing systemic sclerosis. Myocardial biopsy and MRI supported endocardial and myocardial inflammation. Treatment with cyclophosphamide resulted in stabilization of cardiac function and normalization of cardiac enzymes. The patient died due to infectious complications. Case 2, also newly developed systemic sclerosis, presented with renal crisis and pulmonary alveolitis. Elevated cardiac troponin T persisted in the presence of cyclophosphamide treatment, subsequent MRI suggested myocardial inflammation. After stepping up treatment by addition of rituximab cardiac enzymes normalized and cardiac function stabilized.ConclusionWe hypothesize that low-grade endocardial and myocardial inflammation may be more relevant in systemic sclerosis than appreciated previously.