Paraneoplastic autoimmune optic neuritis with retinitis defined by CRMP-5-IgG
Paraneoplastic autoimmune optic neuritis with retinitis defined by CRMP-5-IgG
复制标题
DOI：
10.1002/ana.10587
复制
发表时间：
2003-07-01
影响因子：
11.2
通讯作者：
Lennon, VA
中科院分区：
文献类型：
--
作者：
Cross, SA;Salomao, DR;Lennon, VA
自身抗体确定了两种与小细胞相关的副肿瘤视觉疾病。肺癌：视网膜病变（“CAR”-IgG [23kDa，恢复]）和视神经炎（塌陷反应介质蛋白-5 [CRMP]-IgG [62kDa]）。在16例伴有CRMP-5-IgG和视神经炎的患者（年龄52-74岁，均为吸烟者，9例女性）中，我们记录了5例并发视网膜炎。没有人有CAR-IgG。15例有亚急性视力丧失、视盘肿胀和视场缺损。椎间盘周围及远处血管渗漏明显；5/5的受试者视网膜电图异常。9例玻璃体细胞突出。玻璃体切除术显示反应性淋巴细胞增多（4/4），以CD4（+）为主（1/1）。多数患者伴有多灶性神经系统病变。脑脊液中含有淋巴细胞（7-32）、蛋白升高、多个寡克隆免疫球蛋白带和CRMP-5-IgG。3例患者在发病时表面与德维克病相似。1例尸检患者以CD8(+) T淋巴细胞浸润视神经和脊髓为主。11例患者确诊为小细胞。癌;我有肺癌的影像学证据，3人有肾癌或甲状腺癌。Western blot检测正常视网膜和视神经中CRMP-5蛋白的表达。感光细胞、视网膜神经节细胞和神经纤维表现出crmp -5特异性免疫反应性。综上所述，CRMP-5-IgG定义了一种伴玻璃体炎性细胞的视神经炎和视网膜炎合并的副肿瘤眼科实体。血清学阳性避免了玻璃体活检的需要，并加快了癌症的搜索。
Autoantibodies have defined two paraneoplastic visual disorders related to small-cell. lung carcinoma: retinopathy ("CAR"-IgG [23kDa, recoverin]) and optic neuritis (collapsin response-mediator protein-5 [CRMP]-IgG [62kDa]). Among 16 patients with CRMP-5-IgG and optic neuritis (aged 52-74 years; all smokers, 9 women), we documented coexisting retinitis in 5. None had CAR-IgG. Fifteen had subacute vision loss, swollen optic discs, and field defects. Vascular leakage was evident at and remote from the disc; 5/5 tested had abnormal electroretinograms. Nine had striking vitreous cells. Vitrectomy showed reactive lymphocytosis (4/4), predominantly CD4(+) (1/1). Most patients had multifocal neurological accompaniments. Cerebrospinal fluid contained lymphocytes (7-32), elevated protein, multiple oligoclonal immunoglobulin bands, and CRMP-5-IgG. Three patients superficially resembled Devic's disease at presentation. One autopsied patient had predominantly CD8(+) T lymphocytes infiltrating optic nerve and spinal cord. Eleven patients had confirmed small-cell. carcinoma; I had imaging evidence of lung cancer, 3 had renal or thyroid carcinoma. Full-length CRMP-5 protein was identified in normal retina and optic nerve by Western blot analyses. Photoreceptor cells, retinal ganglion cells, and nerve fibers exhibited CRMP-5-specific immunoreactivity. In summary, CRMP-5-IgG defines a paraneoplastic ophthalmological entity of combined optic neuritis and retinitis with vitreous inflammatory cells. Positive serology obviates the need for vitreous biopsy and expedites the search for cancer.