Department of Internal Medicine and Rheumatology, Justus-Liebig-University of Giessen, Kerckhoff-Klinik, Rheumatology and Clinical Immunology, Benekestr 2-8, 61231 Bad Nauheim, Germany.
Joint Bone Spine. 2013 May;80(3):320-3. doi: 10.1016/j.jbspin.2012.10.009. Epub 2012 Dec 10.
Myocardial 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 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.
We hypothesize that low-grade endocardial and myocardial inflammation may be more relevant in systemic sclerosis than appreciated previously.
系统性硬化症的心肌表现主要归因于纤维性重塑。我们报告了两例病例，其中心内膜和心肌炎症可能是心脏疾病的一个相关组成部分。
病例 1 表现为暴发性三尖瓣衰竭，无肺动脉高压，且新出现系统性硬化症。心肌活检和 MRI 支持心内膜和心肌炎症。环磷酰胺治疗导致心功能稳定和心肌酶正常化。患者因感染并发症死亡。病例 2 也新出现系统性硬化症，表现为肾危象和肺泡炎。环磷酰胺治疗时，心肌肌钙蛋白 T 持续升高，随后 MRI 提示心肌炎症。加用心肌炎治疗后，心肌酶正常化，心功能稳定。
我们假设，低水平的心内膜和心肌炎症可能比以前认为的更为普遍存在于系统性硬化症中。
