Kühnl P, Sibrowski W, Kalmar G, Holzmann H, Sollberg S, Böhm B O
Abt. Transfusionsmedizin u. Transplantationsimmunologie, Universitäts-Krankenhaus Eppendorf.
Beitr Infusionsther. 1990;26:287-9.
A possible HLA disease association was investigated in 40 patients (38 female, 2 male) with progressive systemic sclerosis (PSS) and 42 patients (32 female, 10 male) with morphea. The HLA A, B, C, DR phenotypes of patients were compared with 193 healthy controls. The following relative risk (RR) values were determined in PSS: A1 (1.38), A2 (1.39), B8 (1.67), B15 (3.22), DRw8 (6.30) and in morphea patients: A3 (1.43), B7 (1.39), B40 (1.81), Bw60 (2.49), DR2 (2.38) and DRw8 (2.55), indication a relatively weak, HLA-linked genetic predisposition for the manifestation of the disease. The HLA "risk" antigens for the two clinically different subtypes of the disease are also different: raised A1/B8 frequencies, like in our PSS group, correlate with a high, pathological immune response (autoimmune disorders). In contrast, A3/B7/DR2 prevalence, like in our morphea group, correlates with a low immune response. HLA typing may contribute to clinical differential diagnosis and possibly also prognosis.
对40例进行性系统性硬化症（PSS）患者（38例女性，2例男性）和42例局限性硬皮病患者（32例女性，10例男性）的HLA疾病关联进行了研究。将患者的HLA A、B、C、DR表型与193名健康对照进行比较。在PSS中确定了以下相对风险（RR）值：A1（1.38）、A2（1.39）、B8（1.67）、B15（3.22）、DRw8（6.30）；在局限性硬皮病患者中确定的RR值为：A3（1.43）、B7（1.39）、B40（1.81）、Bw60（2.49）、DR2（2.38）和DRw8（2.55），表明该疾病表现存在相对较弱的HLA相关遗传易感性。该疾病两种临床不同亚型的HLA“风险”抗原也不同：如在我们的PSS组中，A1/B8频率升高与高病理性免疫反应（自身免疫性疾病）相关。相比之下，如在我们的局限性硬皮病组中，A3/B7/DR2的患病率与低免疫反应相关。HLA分型可能有助于临床鉴别诊断，也可能有助于预后判断。
