Engel A G, Arahata K
Hum Pathol. 1986 Jul;17(7):704-21. doi: 10.1016/s0046-8177(86)80180-0.
Monoclonal antibodies reactive for B cells, T cells, T-cell subsets, killer (K) and natural killer (NK) cells, and the Ia antigen were used to analyze mononuclear cell subsets in scleroderma (SD), dermatomyositis (DM), polymyositis (PM), inclusion body myositis (IBM), Duchenne dystrophy (DD), and normal muscle. The analysis, which was quantitative, was performed according to diagnosis and site of accumulation. Cells at perivascular, perimysial, and endomysial sites of accumulation, and cells focally surrounding and invading nonnecrotic muscle fibers, were analyzed separately. Individual antigens were localized in 2-micron serial sections, or multiple antigens were demonstrated in a given section by sequential paired immunofluorescence. The latter approach allowed the identification of the cell phenotypes in which functional properties are defined by multiple markers, e.g., T8+ and T4+ cells that are either activated or not activated, T8+ cells that are either cytotoxic or suppressor T cells, and K/NK cells of varying maturity and killing capability. The interactions of inflammatory cells of various types with each other and the muscle fiber were further investigated by immunoelectron microscopy. In SD, the findings provide evidence for a cell-mediated immune effector response against a connective tissue and/or vascular element. In DM, the effector response appears to be predominantly humoral. In PM and IBM (but not in DM or SD), there is invasion and destruction of nonnecrotic muscle fibers by cytotoxic T cells, with or without accompanying macrophages. Because T-cell-mediated injury is antigen- and major histocompatibility complex-restricted, clones of T cells must have been sensitized previously to a muscle fiber-associated surface antigen. The identity of the putative antigen(s) remains an important, unsolved question.
用针对B细胞、T细胞、T细胞亚群、杀伤（K）细胞和自然杀伤（NK）细胞以及Ia抗原的单克隆抗体来分析硬皮病（SD）、皮肌炎（DM）、多发性肌炎（PM）、包涵体肌炎（IBM）、杜兴肌营养不良症（DD）和正常肌肉中的单核细胞亚群。该分析是定量的，根据诊断和积聚部位进行。分别分析血管周围、肌束膜和肌内膜积聚部位的细胞，以及局灶性围绕和侵入非坏死肌纤维的细胞。将单个抗原定位在2微米连续切片中，或通过顺序配对免疫荧光在给定切片中显示多种抗原。后一种方法可以鉴定由多种标志物定义功能特性的细胞表型，例如活化或未活化的T8 +和T4 +细胞、细胞毒性或抑制性T细胞的T8 +细胞以及具有不同成熟度和杀伤能力的K / NK细胞。通过免疫电子显微镜进一步研究了各种类型的炎症细胞彼此之间以及与肌纤维的相互作用。在硬皮病中，研究结果为针对结缔组织和/或血管成分的细胞介导的免疫效应反应提供了证据。在皮肌炎中，效应反应似乎主要是体液性的。在多发性肌炎和包涵体肌炎中（但在皮肌炎或硬皮病中不是），细胞毒性T细胞会侵入并破坏非坏死肌纤维，有无巨噬细胞伴随。由于T细胞介导的损伤受抗原和主要组织相容性复合体限制，T细胞克隆此前必定已对肌纤维相关表面抗原致敏。推定抗原的身份仍然是一个重要的未解决问题。
