Bae Sangmee, Khanlou Negar, Charles-Schoeman Christina
University of California, Los Angeles Department of Medicine Division of Rheumatology, Los Angeles, CA.
University of California, Los Angeles Department of Pathology and Laboratory Medicine, Los Angeles, CA.
Hum Pathol (N Y). 2017 Jun;8:55-58. doi: 10.1016/j.ehpc.2017.03.005. Epub 2017 Mar 31.
Cardiac involvement has been well recognized in patients with dermatomyositis (DM) and polymyositis (PM) with a variable frequency between 9 and 72%. However, clinically significant heart involvement in DM/PM is relatively infrequent and there have been rare reports of cardiac transplantation in DM. Our aims were to describe a case of severe cardiac involvement in DM requiring heart transplantation and review the literature of cardiac disease in DM and PM.
A patient with dermatomyositis who was referred to our institution with severe heart failure is described. Pathology of the patient's skeletal and cardiac muscle is reviewed. A MEDLINE database search of reports of cardiac involvement in DM and PM was also conducted.
A 36 year-old man with DM presented with severe heart failure to our institution for evaluation of heart transplantation. After a three month hospitalization he underwent successful cardiac transplantation. Pathological examination of his explant heart revealed a pattern of inflammation and damage similar to DM in skeletal muscle. The patient is currently doing well, 20 months post-transplant, and is maintained on tacrolimus, cellcept, rituximab, and low dose prednisone. To our knowledge, this is the first case report of heart transplantation in dermatomyositis in which the muscle pathology is similar in both heart and skeletal muscle.
Severe cardiac involvement requiring transplantation is rare in dermatomyositis but does occur and appears to be related to a similar inflammatory process as noted in the skeletal muscle.
皮肌炎（DM）和多发性肌炎（PM）患者的心脏受累已得到充分认识，其发生率在9%至72%之间不等。然而，DM/PM患者临床上显著的心脏受累相对较少见，且DM患者心脏移植的报道也很罕见。我们的目的是描述一例因严重心脏受累而需要进行心脏移植的DM病例，并回顾DM和PM中心脏疾病的相关文献。
描述了一名因严重心力衰竭转诊至我院的皮肌炎患者。回顾了该患者骨骼肌和心肌的病理情况。还对MEDLINE数据库中有关DM和PM心脏受累报道进行了检索。
一名36岁的DM男性因严重心力衰竭到我院评估心脏移植。经过3个月的住院治疗，他成功接受了心脏移植。对其切除心脏的病理检查显示，炎症和损伤模式与骨骼肌中的DM相似。该患者目前在移植后20个月情况良好，服用他克莫司、骁悉、利妥昔单抗和低剂量泼尼松维持治疗。据我们所知，这是首例皮肌炎心脏移植病例报告，其心脏和骨骼肌的肌肉病理相似。
皮肌炎中需要进行移植的严重心脏受累情况罕见，但确实会发生，且似乎与骨骼肌中观察到的类似炎症过程有关。
