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自身免疫性视网膜病变:具有抗恢复蛋白免疫反应性和全视网膜变性的患者。

Autoimmune retinopathy: patients with antirecoverin immunoreactivity and panretinal degeneration.

作者信息

Heckenlively J R, Fawzi A A, Oversier J, Jordan B L, Aptsiauri N

机构信息

Jules Stein Eye Institute, University of California-Los Angeles School of Medicine, 90095, USA.

出版信息

Arch Ophthalmol. 2000 Nov;118(11):1525-33. doi: 10.1001/archopht.118.11.1525.

DOI:10.1001/archopht.118.11.1525
PMID:11074809
Abstract

PURPOSE

To investigate whether antirecoverin antibodies are present in patients with retinitis pigmentosa (RP). Recoverin, a retinal protein, has been implicated as a cause of cancer-associated retinopathy (CAR), which manifests as an RP-like retinal degeneration. The rationale is that the ocular findings in CAR syndrome are similar to those found in many forms of RP, and since 40% of patients with RP have no family history, some patients may have an underlying autoimmune process causing or contributing to their retinopathy.

METHODS

Serum samples from 521 patients diagnosed with RP were screened for antiretinal proteins activity by Western blot analysis. Fifty-one patients had antibody reactivity against retinal proteins in the range of 23 to 26 kd and underwent dot-blot analysis for antirecoverin antibody, checking IgG and IgM antibodies. Enzyme-linked immunosorbent assay (ELISA) was performed to evaluate the titer of antirecoverin antibodies in patients with positive results on dot-blot analysis. Lymphocyte proliferation assays using recoverin were performed on 26 samples.

RESULTS

Ten patients were found to have antirecoverin antibody and/or cellular immunoreactivity. Eight patients had positive dot-blot testing: 6 patients had both IgG and IgM antirecoverin activity, and 1 patient each had IgG or IgM activity. In these 8 patients, numerous other antiretinal protein antibodies were present. Three patients had positive recoverin-mediated lymphocyte proliferation, and all patients were positive for antirecoverin antibodies on ELISA testing.

CONCLUSIONS

Antirecoverin immunoreactivity was found in 10 patients without systemic malignancy but with clinical findings consistent with RP. These results suggest that there are other immunogenic mechanisms occurring in the formation of antirecoverin antibodies in addition to the putative tumor-mediated mechanisms. This survey suggests that there may be rare cases of CAR-like syndrome in the category of simplex RP, or that some patients with RP also have antirecoverin antibodies that may be exacerbating their underlying disease. Arch Ophthalmol. 2000;118:1525-1533

摘要

目的

研究视网膜色素变性(RP)患者体内是否存在抗恢复蛋白抗体。恢复蛋白是一种视网膜蛋白,被认为是癌症相关性视网膜病变(CAR)的病因,CAR表现为类似RP的视网膜变性。理论依据是CAR综合征的眼部表现与许多形式的RP相似,且由于40%的RP患者无家族病史,一些患者可能存在潜在的自身免疫过程导致或促成其视网膜病变。

方法

通过蛋白质印迹分析对521例诊断为RP的患者血清样本进行抗视网膜蛋白活性筛查。51例患者对23至26kd范围内的视网膜蛋白具有抗体反应性,并接受了抗恢复蛋白抗体的斑点印迹分析,检测IgG和IgM抗体。对斑点印迹分析结果呈阳性的患者进行酶联免疫吸附测定(ELISA)以评估抗恢复蛋白抗体的滴度。对26份样本进行了使用恢复蛋白的淋巴细胞增殖试验。

结果

发现10例患者具有抗恢复蛋白抗体和/或细胞免疫反应性。8例患者斑点印迹检测呈阳性:6例患者同时具有IgG和IgM抗恢复蛋白活性,1例患者分别具有IgG或IgM活性。在这8例患者中,还存在许多其他抗视网膜蛋白抗体。3例患者恢复蛋白介导的淋巴细胞增殖呈阳性,且所有患者ELISA检测抗恢复蛋白抗体均为阳性。

结论

在10例无全身恶性肿瘤但临床症状与RP一致的患者中发现了抗恢复蛋白免疫反应性。这些结果表明,除了假定的肿瘤介导机制外,抗恢复蛋白抗体形成过程中还存在其他免疫原性机制。这项调查表明,单纯性RP类别中可能存在罕见的CAR样综合征病例,或者一些RP患者也具有抗恢复蛋白抗体,这可能会加重其潜在疾病。《眼科学文献》。2000年;118:1525 - 1533

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