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无抗核抗体或其他自身抗体的播散性红斑狼疮

[Disseminated lupus erythematosus without antinuclear antibodies or other autoantibodies].

作者信息

Duc J, Perroulaz G, Golaz J, Campiche M, Pécoud A

出版信息

Schweiz Med Wochenschr. 1987 Feb 7;117(6):205-8.

PMID:3494306
Abstract

Antinuclear antibodies (ANA) are found in the majority of patients with systemic lupus erythematosus (SLE). We report here the only documented case, out of a series of 38 patients, in which SLE was diagnosed in spite of the fact that we failed to demonstrate any type of autoantibodies. A 25-year-old black woman presented with 6 of the 11 criteria of the American Rheumatism Association for classification of SLE, between August 1984 and April 1985, i.e. malar rash, photosensitivity, arthritis, pleurisy and pericarditis, renal insufficiency and nephrotic syndrome, anemia and leukopenia. Renal biopsy revealed mesangial glomerulonephritis, tubulonephritis and many tubuloreticular inclusions in the capillary endothelium highly suggestive of SLE. Four ANA determinations were performed during the 8 months of observation which were all negative, as were all other antibodies (anti-nDNA, -Sm, -RPN, -Ro, -La). The outcome was very favourable under prednisone and cyclophosphamide. In the rare cases of ANA negative SLE (5-10%) photosensitive dermatitis is the prominent feature and renal or central nervous system involvement is less frequent. Those patients usually have other types of autoantibodies (especially anticytoplasmic) which was not the case in our patient. This indicates that the absence of autoantibodies does not rule out SLE.

摘要

抗核抗体(ANA)在大多数系统性红斑狼疮(SLE)患者中均可检测到。我们在此报告了38例患者中的唯一一例有记录的病例,尽管我们未能检测到任何类型的自身抗体,但该患者仍被诊断为SLE。一名25岁的黑人女性在1984年8月至1985年4月期间出现了美国风湿病协会用于SLE分类的11项标准中的6项,即颧部红斑、光过敏、关节炎、胸膜炎和心包炎、肾功能不全和肾病综合征、贫血和白细胞减少。肾活检显示系膜增生性肾小球肾炎、肾小管肾炎以及毛细血管内皮中有许多管网状包涵体,高度提示为SLE。在8个月的观察期内进行了4次ANA检测,结果均为阴性,其他所有抗体(抗双链DNA、抗Sm、抗RNP、抗Ro、抗La)检测结果也均为阴性。在泼尼松和环磷酰胺治疗下,病情转归非常良好。在罕见的ANA阴性SLE病例(5%-10%)中,光敏性皮炎是突出特征,肾脏或中枢神经系统受累较少见。这些患者通常有其他类型的自身抗体(尤其是抗细胞质抗体),而我们的患者并非如此。这表明自身抗体的缺乏并不能排除SLE。

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