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家族性复发性风湿病:与人类白细胞抗原的可能关联。

Familial palindromic rheumatism: a possible association with HLA.

作者信息

Pines A, Kaplinsky N, Orgad S, Gazit E, Frankl O

出版信息

Ann Rheum Dis. 1983 Dec;42(6):631-3. doi: 10.1136/ard.42.6.631.

Abstract

Palindromic rheumatism is very rare in children. The familial occurrence of the disease has been only briefly reported previously. A family is described here in which the mother and her 3 children suffered from typical palindromic rheumatism, which ran a benign course. All 4 members were seronegative and the HLA types of the children were identical. The HLA genotype of the mother was HLA A2, Cw4, Bw41, Bw6, DR5, MT2/Aw32, CX, Bw44, Bw4, DR1, MT1. HLA DR5 has previously been found to be associated with juvenile rheumatoid arthritis, while DR1 in Jews is significantly associated with adult-onset rheumatoid arthritis. DR5 was shared by the mother and her 3 children. The presence of the antigen DR5 in this sibship suggests that palindromic rheumatism may be a variant of juvenile rheumatoid arthritis with a relatively late onset and a more benign course. Tissue typing of patients with palindromic rheumatism may give a clue to prognosis. The patients' youth and the familial involvement, with identical HLA characteristics, are the outstanding features of this family.

摘要

回纹型风湿症在儿童中非常罕见。此前仅有关于该疾病家族性发病的简短报道。本文描述了一个家庭,母亲及其3个孩子均患有典型的回纹型风湿症,病情呈良性经过。所有4名家庭成员血清学检查均为阴性,孩子们的HLA类型相同。母亲的HLA基因型为HLA A2、Cw4、Bw41、Bw6、DR5、MT2/Aw32、CX、Bw44、Bw4、DR1、MT1。此前发现HLA DR5与青少年类风湿关节炎相关,而在犹太人中DR1与成人型类风湿关节炎显著相关。母亲及其3个孩子均具有DR5抗原。在这个家族中DR5抗原的存在提示回纹型风湿症可能是青少年类风湿关节炎的一种变异型,起病相对较晚,病程较为良性。对回纹型风湿症患者进行组织分型可能有助于判断预后。患者的年轻以及家族性受累且具有相同的HLA特征是这个家庭的突出特点。

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