体液细胞免疫在进行性肌营养不良症发病过程中的作用

Effects of humoral and cellular immunity in pathologic progress in PMD by immunohistochemistry

探讨体液免疫和细胞免疫在进行性肌营养不良症（ＰＭＤ）发病过程中的作用。方法应用免疫组化ＳＰ法及免疫荧光一步法。结果ＰＭＤ肌组织中免疫球蛋白ＩｇＭ、ＩｇＧ和补体Ｃ３的阳性表达率分别为５０．０％、３１．１％和１１．１％，ＩｇＭ与对照组相比，差异有显著性意义（Ｐ＜０．０５），主要定位于肌膜和间质小血管壁上，与肌纤维萎缩性病变有关；ＰＭＤ肌组织中有巨噬细胞和Ｔ淋巴细胞浸润，阳性反应率分别为１００．０％和５５．５％，巨噬细胞聚集在坏死灶中，Ｔ细胞分布于退变肌纤维和血管周围，以ＣＤ８＋Ｔ细胞为主，多数表达ＨＬＡ－ＤＲ。结论提示免疫因素可能参与ＰＭＤ的病变过程。

Objective To study the effects of humoral and cellular immunity in pathologic progress in progressive muscular dystrophies (PMD). Methods Humoral and cellular immunity were studied in pathologic progress from 18 cases of PMD by immunohistochemistry (SP) and direct immunofluscence, and 11 cases of non muscular desease, nine cases of normal muscle were as controls. Result The positive rates of IgM, IgG and C3 were detected as 50.0%, 31.1%, and 11.1% respectively in PMD. Statistically significant IgM reaction was deposited as compared with controls ( P <0.05). The immune complex of positive reaction mostly located on the sarcolema and blood vessel walls, and were related to atrophy of myofibers. The positive rates of macrophage cells and T lymphocytes with infiltrated on muscle tissue were detected as 100.0% and 55.5% respectively. The macrophages mainly distributed to the necrosis lesions, while T cells mostly CD8 + surrounded degenerating myofibers and blood vessels and expressed HLA DR. Conclusion The results suggested that immunitive factor might be involved in pathologic progress in PMD.