Walter P, Weill-Bousson M, Mossart J M, Voegtlin R
Virchows Arch A Pathol Anat Histol. 1977 Aug 9;375(1):71-82. doi: 10.1007/BF00430645.
Histological and ultrastructural studies were performed on myocardial biopsies and aortic and mitral valve leaflets obtained during an operation on a patient with Systemic Lupus Erythematosus (S.L.E.). Congestive heart failure and valvular dysfunction appeared five years after the diagnosis of S.L.E. was made. On histological study, aortic and mitral valve leaflets are uniformly thickened by fibrous tissue with a nodular appearance. No active endocarditis was associated with the fibrous scarring. Atrial myocardium and papillary muscle countain a fibrous net-work discret in the former, extensive in the latter. The scattered foci of fibrosis in the papillary muscle surround vessels without obliteration or parietal necrosis. Ultrastructurally their lumina appears narrowed by prominent endothelial cells with cytoplasmic aggregates of tubuloreticular structures (T.R.S.). These tubules are also present in some endocardial endothelial cells but are rare in the normal intrapapillary or atrial vessels that are not associated with a scar. Myocardial fibrous foci enclose atrophic and severely degenerated cardiac muscle cells; other cells situated at the periphery of the foci are normal in size or hypertrophic and moderately degenerated. The most altered muscle cells show an important loss of myofibrils, a proliferation of sarcoplasmic reticulum in myofibril free spaces, or necrosis with macrophagic resorption. Focal changes with loss of myofilaments, Z material streaming and concentric lamellar bodies are found in moderately degenerate cardiac muscle cells. The remaining papillary muscle cells and the atrial cells are all hypertrophied without degeneration. These changes suggest that focal myocardial fibrosis and associated cardiac muscle cell degeneration may be responsible for impaired cardiac performance in some patients with S.L.E. According to the constant topographic relation between the narrowed vessels whose endothelial cells contain T.R.S. and the surrounding fibrous foci, we believe that the myocardial fibrous patches may correspond to scarring of microinfarcts related to active S.L.E. vascularitis.
对一名系统性红斑狼疮（S.L.E.）患者手术期间获取的心肌活检组织以及主动脉瓣和二尖瓣小叶进行了组织学和超微结构研究。在S.L.E.诊断五年后出现了充血性心力衰竭和瓣膜功能障碍。组织学研究显示，主动脉瓣和二尖瓣小叶均被呈结节状外观的纤维组织均匀增厚。纤维瘢痕形成未伴有活动性心内膜炎。心房心肌和乳头肌含有纤维网络，前者分散，后者广泛。乳头肌中的散在纤维化灶围绕血管，无管腔闭塞或血管壁坏死。超微结构显示，其管腔因突出的内皮细胞而变窄，内皮细胞含有管状网状结构（T.R.S.）的细胞质聚集体。这些小管也存在于一些心内膜内皮细胞中，但在与瘢痕无关的正常乳头内或心房血管中很少见。心肌纤维灶包围着萎缩和严重变性的心肌细胞；位于病灶周边的其他细胞大小正常或肥大且中度变性。变化最明显的肌肉细胞显示肌原纤维大量丢失、肌原纤维间隙中肌浆网增生或伴有巨噬细胞吸收的坏死。在中度变性的心肌细胞中发现了伴有肌丝丢失、Z物质流和同心层状体的局灶性变化。其余的乳头肌细胞和心房细胞均肥大但未变性。这些变化表明，局灶性心肌纤维化和相关的心肌细胞变性可能是一些S.L.E.患者心脏功能受损的原因。根据内皮细胞含有T.R.S.的狭窄血管与周围纤维灶之间恒定的地形关系，我们认为心肌纤维斑块可能对应于与活动性S.L.E.血管炎相关的微梗死瘢痕。
