Elchidiac A, Lepage V, Berrih S, Colombani J, Degos L
Pathol Biol (Paris). 1985 Jan;33(1):50-4.
Genetic susceptibility (HLA types), clinical and pathological findings, amount of acetylcholine receptor antibodies and T lymphocyte subpopulations were studied in 63 patients with Myasthenia Gravis (MG). The frequency of HLA-DR5 was increased among patients (0.50 versus 0.23 in controls, pc less than 0.01, relative risk 3.3) and that of HLA-DR3 previously described as associated with MG was slightly increased (0.31 versus 0.20 in controls). The relative frequencies of two T cell subpopulations (T4 helper and T8 suppressor/cytotoxic lymphocytes) were normal in HLA-DR5 positive patients while the ratio T4/T8 was increased in other MG patients, who were HLA-DR3 (p less than 0.005). The high rate was due to an increase in the absolute number of T4 lymphocytes (p less than 0.001). HLA-DR3 patients were mostly women with early onset of a severe form of the disease, marked by the presence of thymic follicular lymphoid hyperplasia. A third genetic susceptibility to this disease was recently described in patients treated with D-penicillamine, the antigenic frequency of HLA-Bw35, DR1 is significantly increased. These 3 types of association between HLA and myasthenia gravis can be related to three different physiopathological mechanisms: the first two are probably linked to individual immunity (inductor/suppressor disequilibrium), in the third association, the mechanism is immunopharmacological.
对63例重症肌无力(MG)患者的遗传易感性(HLA类型)、临床和病理表现、乙酰胆碱受体抗体量及T淋巴细胞亚群进行了研究。患者中HLA - DR5的频率增加(患者为0.50,对照组为0.23,pc小于0.01,相对风险3.3),先前描述的与MG相关的HLA - DR3频率略有增加(对照组为0.20,患者为0.31)。在HLA - DR5阳性患者中,两个T细胞亚群(T4辅助性和T8抑制性/细胞毒性淋巴细胞)的相对频率正常,而在其他MG患者(HLA - DR3)中,T4/T8比值增加(p小于0.005)。该高比值是由于T4淋巴细胞绝对数量增加所致(p小于0.001)。HLA - DR3患者大多为女性,疾病早期起病,病情严重,以胸腺滤泡性淋巴样增生为特征。最近在接受D - 青霉胺治疗的患者中描述了对该疾病的第三种遗传易感性,HLA - Bw35、DR1的抗原频率显著增加。HLA与重症肌无力之间的这3种关联类型可能与3种不同的生理病理机制有关:前两种可能与个体免疫(诱导/抑制失衡)有关,在第三种关联中,机制为免疫药理学。