全身性紅斑性狼瘡病人之脊髓炎

Myelitis in patients with systemic lupus erythematosus

目的：探討紅斑性狼瘡脊髓炎的臨床表現、治療、及癒後。方法：分析高雄長庚醫院1998至2009年間，收錄符合美國風濕病學會全身性紅斑性狼瘡準則，且須發生脊髓炎的病人為研究對象。共收錄10位全身性紅斑性狼瘡併脊髓炎病人。結果：本族群中，有80%病人為女姓，中位數年齡為39.5歲。臨床表現為，90%病人有感覺異常，80%病人有下肢無力，80%病人有括約肌功能障礙。30%病人先被診斷為尿道感染而住院。88%病人脊髓病灶於頸椎至上胸椎間。治療方式包括傳統高劑量類固醇、脈衝類固醇、cyclophosphamide、血漿置換，60%病人癒後不佳。五位病人治療方式中，包含了cyclophosphamide，其中60%得到較好的癒後。結論：全身性紅斑性狼瘡患者，發生脊髓炎時，常表現解尿困難徵狀，而以尿道感染的診斷來住院。而最常出現病灶的位置為頸椎至上胸椎。治療方面，高劑量類固醇併用cyclophosphamide可能有較好的癒後。

Objective: Myelitis is a rare but severe neurologic presentation of patients with systemic lupus erythematosus (SLE). Few large-scale retrospective cohort studies have been conducted, especially in Taiwan; therefore, we designed a retrospective study to investigate the disease parameters, treatment, and prognosis of myelitis in southern Taiwan. Methods: We reviewed medical records of patients with SLE who were evaluated at the Chang Gung Memorial Hospital-Kaohsiung Medical Center between January 1998 and January 2009. A total of 10 patients with myelopathy were included in the study. Neurologic variables and serologic features of SLE were assessed. Magnetic resonance images (MRI) of the spine and cerebrospinal fluid profiles were collected. We also analyzed the treatment and outcome of myelitis. Results: The cohort of 10 patients included 8 females (80%) and 2 males (20%). Three patients (30%) were initially admitted under the tentative impression of urinary tract infection (UTI). In 8 patients diagnosed with myelitis using MRI, 7 patients (88%) had increased T2 MRI signal intensity over the cervical and upper thoracic spinal area. Treatment regimens included conventional high-dose glucocorticoid, pulse methylprednisolone, cyclophosphamide, and plasma exchange. Six patients (60%) had a poor outcome. Additional cyclophosphamide was used in 5 patients and 3 of them (60%) had a good outcome. Conclusion: SLE patients, who contracted myelitis, presented with possible urinary difficulty and were admitted under the tentative impression of UTI. Besides, the most frequent site of lupus myelitis is the cervical to upper thoracic spinal area. Treatment with high-dose glucocorticoid and cyclophosphamide may be related to a better outcome compared to those without using cyclophosphamide.