臺灣漢人族群紅斑性狼瘡病患併發橫斷性脊髓炎之研究

Investigation of transverse myelitis in systemic lupus erythematosus patients, a Taiwanese experience

目的：橫斷性骨髓炎是紅斑性狼瘡的神經學表現之ㄧ，可能造成長期神經學後遺症甚至導致死亡；然而，只有少部分研究描述其特徵。此研究提供了臺灣漢人族群紅斑性狼瘡病患合併橫斷性骨髓炎的資料。方法：我們納入了12位紅斑性狼瘡病患，其於西元2002年7月至 2012年6月間橫斷性骨髓炎共發作17次，我們將回溯其醫療紀錄以獲得人口學資料、臨床特徵、實驗室數據以及影像學檢查結果，並使用擴展殘疾狀況評分表（Expanded Disability Status Scale）評估橫斷性骨髓炎疾病嚴重度。結果：納入的12名病患（2名男性及10名女性）年齡中位數為39歲；17次橫斷性骨髓炎發作中64.7%的紅斑性狼瘡疾病活性指數（Systemic Lupus Erythematosus Disease Activity Index）改變大於3分，代表同時紅斑性狼瘡疾病的復發。高比例的病患腦脊液中的免疫球蛋白G指標（IgG index）或蛋白質升高；而大多數發作（83.3%）可在脊髓核磁共振攝影上發現大於4節的病灶。比較特別的是，只有8.3 % 的病患檢測到抗磷酯抗體，而且沒有任何病患產生抗磷酯抗體症候群。橫斷性骨髓炎疾病嚴重度與腦脊液白血球量以及同時或曾經表現過的血小板低下相關；病患經治療六個月後的擴展殘疾狀況評分表分數只有些微進步。結論：在臺灣漢人族群中，橫斷性骨髓炎的許多特徵與高加索人種相似，常可觀察到全身性的紅斑性狼瘡疾病復發。與高加索人相比，抗磷酯抗體可能在致病機轉上扮演較不重要的角色。

Objective: Transverse myelitis (TM) is one of the neurological manifestations of systemic lupus erythematosus (SLE). It can bring about long-term neurological sequelae and even mortalities. However, only a few studies have described its characteristics. This study provided data in Taiwanese Han SLE patients with TM. Methods: We recruited 12 SLE patients with 17 episodes of transverse myelitis from July 2002 to June 2012 and reviewed their medical records. Demographic data, clinical characteristics, laboratory findings and radiographic features were obtained. Severity of TM was expressed in Expanded Disability Status Scale (EDSS) scoring. Results: The median age of the 12 patients (two males and ten females) was 34 years. Among the 17 TM episodes, there was a greater than 3-point change in SLEDAI score in 64.7%, indicating a lupus flare. Most of patients had either elevated cerebrospinal fluid IgG index or protein. Involvement of more than 4 segments in spinal MRI was observed in most episodes (83.3%) of TM. Antiphospholipid antibodies were detected in only 8.3% of our patients and no case of antiphospholipid antibody syndrome was documented. More severe disease was associated with increased white blood cell count in cerebrospinal fluid and thrombocytopenia, manifested concomitant with or before the episode of TM. There was only modest improvement after treatment 6 months later in terms of EDSS scores. Conclusion: Among the Taiwanese Han population, TM is similar to that in Caucasians in many aspects. Generalized lupus flare is often present. Antiphospholipid antibodies may play a minor role in TM pathogenesis in Taiwanese SLE patients, in contrast to Caucasians with SLE-related TM.