Przybojewski J Z, Mynhardt J H, van der Walt J J, Tiedt F A
S Afr Med J. 1985 Oct 26;68(9):680-6.
A 27-year-old black woman with cardiac failure, angina pectoris and Raynaud's syndrome is presented. Skin biopsy and barium studies established the diagnosis of scleroderma (progressive systemic sclerosis (PSS)). Systemic lupus erythematosus (SLE) was strongly suggested by the results of immunological studies and increasing severity of renal failure. Because of the possibility of a cardiomyopathy, cardiac catheterization, selective coronary angiography and right ventricular endomyocardial biopsy were carried out but failed to show any histological features of either SLE or PSS. The patient went into progressive renal failure despite immunosuppressive therapy and plasmapheresis and died; consent for autopsy was refused. A final diagnosis of mixed connective tissue disease (MCTD) was made. The salient features of cardiac involvement in SLE, PSS and MCTD are outlined.
本文报道了一名27岁的黑人女性，患有心力衰竭、心绞痛和雷诺综合征。皮肤活检和钡剂检查确诊为硬皮病（进行性系统性硬化症（PSS））。免疫学研究结果及肾衰竭病情加重强烈提示系统性红斑狼疮（SLE）。由于存在心肌病的可能，进行了心导管检查、选择性冠状动脉造影和右心室心内膜活检，但未发现SLE或PSS的任何组织学特征。尽管进行了免疫抑制治疗和血浆置换，患者仍出现进行性肾衰竭并死亡；家属拒绝尸检。最终诊断为混合性结缔组织病（MCTD）。文中概述了SLE、PSS和MCTD心脏受累的显著特征。
