Neuromyelitis Optica and non-organ-specific Autoimmunity

Neuromyelitis Optica and non-organ-specific Autoimmunity
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DOI:
10.1001/archneurol.2007.17
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发表时间:
2008-01-01
影响因子:
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通讯作者:
Weinshenker, Brian G.
Weinshenker, Brian G.
中科院分区:
其他
文献类型:
--
作者:
Pittock, Sean J.;Lennon, Vanda A.;Weinshenker, Brian G.

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背景:视神经脊髓炎 (NMO) 通常与非器官特异性自身免疫的其他临床或血清学标志物相关。 目的:评估 NMO 谱系疾病 (NMOSD),包括 NMO、纵向广泛性横贯性脊髓炎和复发性视神经炎与自身免疫性疾病之间的关系。我们重点关注与系统性红斑狼疮 (SLE)、干燥综合征 (SS) 或这些疾病的血清学证据的关联,这些疾病通常是诊断混乱的根源。设计:回顾性盲法血清学调查。地点:罗切斯特梅奥诊所医学院和里尔地区大学中心医院。方法:第 1 组包括 153 名患有 NMOSD 的美国患者(78 名患有 NMO,75 名患有纵向广泛横纹)脊髓炎)和 33 名患有 SS/SLE 的对照受试者。第 2 组包括 30 名法国 SS/SLE 患者,14 名患有 NMOSD(6 名患有 NMO,6 名患有纵向广泛横贯性脊髓炎,2 名患有复发性视神经炎),16 名无 NMOSD,4 名患有 NMO 但无 SS/SLE。 结果:第 1 组中,66.7% 检测到 NMO-IgG,43.8% 检测到抗核抗体,并患有 A 型干燥综合征(SSA) 抗体存在于 15.7% 的 NMO 和纵向广泛横贯性脊髓炎患者中。 5 名患有 NMOSD 的 NMO-IgG 血清阳性患者同时患有 SLE、SS 或两者兼有。抗核抗体和 SSA 抗体在 NMO-IgG 血清阳性患者中比在 NMO-IgG 血清阴性患者中更常见 (P=.001)。对于第 2 组,在 14 名患有 NMOSD 和 SS/SLE 的患者中,有 5 名 (35.7%) 检测到了 NMO-IgG,在 4 名患有 NMO 但不伴有 SS/SLE 的患者中,有 2 名 (50.0%) 检测到了 NMO-IgG (P=.59)。我们仅在 NMOSD 患者中检测到 NMO-IgG,而在 2 个队列中的 49 名 SS/SLE 但没有视神经炎或脊髓炎的对照中未检测到 NMO-IgG (P-.01)。结论:视神经脊髓炎谱系疾病,且 NMO-IgG 血清阳性结果与 SS/SLE 或非器官特异性自身抗体同时存在,表明存在 NMO,而不是血管病变或其他并发症。 SS/SLE。
Background: Neuromyelitis optica (NMO) is often associated with other clinical or serological markers of non-organ-specific autoimmunity.Objective: To evaluate the relationship between NMO spectrum disorders (NMOSDs), including NMO, longitudinally extensive transverse myelitis, and recurrent optic neuritis, and autoimmune disease. We concentrated on the association with systemic lupus erythematosus (SLE), Sjogren syndrome (SS), or serological evidence of these disorders, which commonly is a source of diagnostic confusion.Design: Retrospective blinded serological survey.Setting: Mayo Clinic College of Medicine, Rochester, and Centre Hospitalier Regional Universitaire de Lille.Methods: Group 1 included 153 US patients with NMOSDs (78 with NMO and 75 with longitudinally extensive transverse myelitis) and 33 control subjects with SS/SLE. Group 2 included 30 French patients with SS/SLE,14 with NMOSDs (6 with NMO, 6 with longitudinally extensive transverse myelitis, and 2 with recurrent optic neuritis), 16 without NMOSDs, and 4 with NMO without SS/SLE.Results: For group 1, NMO-IgG was detected in 66.7%, antinuclear antibodies in 43.8%, and Sjogren syndrome A (SSA) antibodies in 15.7% of patients with NMO and longitudinally extensive transverse myelitis. Five NMO-IgG-seropositive patients with NMOSDs had coexisting SLE, SS, or both. Antinuclear antibodies and SSA antibodies were more frequent in NMO-IgG-seropositive patients than in NMO-IgG-seronegative patients (P=.001). For group 2, NMO-IgG was detected in 5 of 14 patients (35.7%) with NMOSDs and SS/SLE and in 2 of 4 patients (50.0%) with NMO without SS/SLE (P=.59). We detected NMO-IgG only in patients with NMOSDs and not in 49 controls with SS/SLE but without optic neuritis or myelitis from the 2 cohorts (P-.01).Conclusion: Neuromyelitis optica spectrum disorders with seropositive findings for NMO-IgG occurring with SS/SLE or non-organ-specific autoantibodies is an indication of coexisting NMO rather than a vasculopathic or other complication of SS/SLE.