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多发性肌炎、肺纤维化与氨酰tRNA合成酶自身抗体

Polymyositis, pulmonary fibrosis and autoantibodies to aminoacyl-tRNA synthetase enzymes.

作者信息

Marguerie C, Bunn C C, Beynon H L, Bernstein R M, Hughes J M, So A K, Walport M J

机构信息

Rheumatology Unit, Royal Postgraduate Medical School, Hammersmith Hospital, London.

出版信息

Q J Med. 1990 Oct;77(282):1019-38. doi: 10.1093/qjmed/77.1.1019.

DOI:10.1093/qjmed/77.1.1019
PMID:2267280
Abstract

The clinical and laboratory features of 29 patients who had one of three anti-aminoacyl-tRNA synthetase autoantibodies, anti-Jo1 (histidyl-tRNA synthetase), anti-PL12 (alanyl-tRNA synthetase) or anti-PL7 (threonyl-tRNA synthetase) were analysed and compared with the findings of other published reports. These autoantibodies were found to be associated with a syndrome delineated by inflammatory myositis (24 patients) and pulmonary fibrosis (23 of 29), but also including inflammatory arthritis (26/29), keratoconjunctivitis sicca (17/29), sclerodactyly (21/29), Raynaud's phenomenon (27/29), hepatitis (8/29) and subcutaneous calcinosis (7/29). The most important clinical determinant of outcome in this group of patients was the severity of the interstitial pulmonary disease. No patient fulfilled the classification criteria for systemic lupus erythematosus, although 10 had autoantibodies to extractable nuclear antigens including Ro, La, RNP, and Sm, and two patients had anti-dsDNA antibodies. Although it seems unlikely that anti-aminoacyl-tRNA synthetase antibodies are directly responsible for causing disease, they may provide an important clue to the aetiology of this unusual syndrome.

摘要

分析了29例患有三种抗氨酰tRNA合成酶自身抗体之一(抗Jo1(组氨酰tRNA合成酶)、抗PL12(丙氨酰tRNA合成酶)或抗PL7(苏氨酰tRNA合成酶))患者的临床和实验室特征,并与其他已发表报告的结果进行了比较。发现这些自身抗体与一种由炎性肌炎(24例患者)和肺纤维化(29例中的23例)所界定的综合征相关,但也包括炎性关节炎(26/29)、干燥性角结膜炎(17/29)、指(趾)硬皮病(21/29)、雷诺现象(27/29)、肝炎(8/29)和皮下钙质沉着(7/29)。在这组患者中,疾病转归的最重要临床决定因素是间质性肺疾病的严重程度。尽管有10例患者有针对可提取核抗原(包括Ro、La、RNP和Sm)的自身抗体,2例患者有抗双链DNA抗体,但没有患者符合系统性红斑狼疮的分类标准。虽然抗氨酰tRNA合成酶抗体似乎不太可能直接导致疾病,但它们可能为这种不寻常综合征的病因提供重要线索。

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