Department of Neurology, Mayo Clinic, Rochester, MN.
Department of Neurology, Mayo Clinic, Rochester, MN; Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, MN.
Mayo Clin Proc. 2018 Sep;93(9):1299-1304. doi: 10.1016/j.mayocp.2018.02.006. Epub 2018 Apr 11.
Antiphospholipid (aPL) antibodies have historically been postulated to cause a poorly understood inflammatory myelitis. Neuromyelitis optica spectrum disorder (NMOSD) causes an inflammatory longitudinally extensive transverse myelitis (LETM). In 2004, aquaporin-4 immunoglobulin G (AQP4-IgG) was first reported as a highly specific (>99%) serum diagnostic biomarker of NMOSD, distinguishing it from other disorders (eg, multiple sclerosis). We sought to assess the frequency of AQP4-IgG (and thus NMOSD diagnosis) in LETM with aPL antibodies. We searched Mayo Clinic records (from January 1, 1996, through December 31, 2014) for patients with (1) LETM and (2) aPL or β-glycoprotein I antibodies and (3) a serum sample available. AQP4-IgG was evaluated in the 24 included patients and in 20 controls with aPL antibodies but without myelitis. Seropositivity for AQP4-IgG was confirmed in 11 of 24 patients with LETM (46%), confirming an AQP4-IgG-seropositive NMOSD diagnosis rather than aPL-associated LETM. Six of 11 AQP4-IgG-seropositive patients (54%) were initially diagnosed as having aPL/lupus-associated myelitis. Recurrent LETM was exclusive to AQP4-IgG-seropositive patients (P=.003). Alternative diagnoses assigned to the remaining 13 AQP4-IgG-seronegative patients included idiopathic transverse myelitis (n=5), seronegative NMOSD (n=2), spinal cord infarct attributed to aPL antibodies (n=2), spinal cord sarcoidosis (n=1), varicella-zoster virus myelitis (n=1), postinfectious myelitis (n=1), and multiple sclerosis (n=1). All 20 controls were seronegative for AQP4-IgG. Clotting disorders occurred in 36% of patients (4 of 11) with LETM with both aPL antibodies and AQP4-IgG. AQP4-IgG should be tested in all patients with LETM and aPL antibodies because AQP4-IgG-seropositive NMOSD accounts for almost half of all cases. Clotting disorders are common in patients with LETM with dual positivity for AQP4-IgG and aPL antibodies.
抗磷脂 (aPL) 抗体过去被认为会导致一种机制尚不清楚的炎症性脊髓炎。视神经脊髓炎谱系疾病 (NMOSD) 会引起炎症性长节段横贯性脊髓炎 (LETM)。2004 年，水通道蛋白 4 免疫球蛋白 G (AQP4-IgG) 首次被报道为 NMOSD 的高度特异性 (>99%) 血清诊断生物标志物，可将其与其他疾病（例如多发性硬化症）区分开来。我们试图评估抗磷脂 (aPL) 抗体阳性的 LETM 患者中 AQP4-IgG（因此 NMOSD 诊断）的频率。我们检索了梅奥诊所的记录（1996 年 1 月 1 日至 2014 年 12 月 31 日），寻找以下患者：(1) LETM 和 (2) aPL 或β-糖蛋白 I 抗体和 (3) 有血清样本。在 24 名纳入患者和 20 名有 aPL 抗体但无脊髓炎的对照中评估了 AQP4-IgG。24 名 LETM 患者中有 11 名（46%）抗 AQP4-IgG 阳性，证实了 AQP4-IgG 阳性 NMOSD 诊断，而不是 aPL 相关 LETM。11 名抗 AQP4-IgG 阳性患者中有 6 名（54%）最初被诊断为 aPL/狼疮相关脊髓炎。复发性 LETM 仅发生在抗 AQP4-IgG 阳性患者中（P=.003）。其余 13 名抗 AQP4-IgG 阴性患者的其他诊断包括特发性横贯性脊髓炎 (n=5)、抗 AQP4-IgG 阴性 NMOSD (n=2)、归因于 aPL 抗体的脊髓梗死 (n=2)、脊髓结节病 (n=1)、水痘带状疱疹病毒脊髓炎 (n=1)、感染后脊髓炎 (n=1) 和多发性硬化症 (n=1)。20 名对照均抗 AQP4-IgG 阴性。LETM 伴 aPL 抗体和 AQP4-IgG 的患者中，有 36%（11 名患者中的 4 名）存在凝血障碍。应在所有抗磷脂抗体阳性的 LETM 患者中检测 AQP4-IgG，因为 AQP4-IgG 阳性 NMOSD 占所有病例的近一半。AQP4-IgG 和 aPL 抗体双重阳性的 LETM 患者中，凝血障碍很常见。
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