系统性红斑狼疮合并肢端坏疽的临床表现与危险因素及预后分析

目的探讨系统性红斑狼疮（SLE）患者合并肢端坏疽的临床表现、危险因素及预后。方法回顾性分析1997年12月至2007年8月北京协和医院住院的2684例SLE患者及其中合并肢端坏疽者的病历资料，统计其临床表现、实验室检查及治疗情况；并对18例SLE坏疽患者进行随访。结果SLE合并肢端坏疽患者平均发病年龄（33±12）岁，平均病程（99±60）个月。长病程（≥4年）、雷诺现象、C反应蛋白升高及抗SSA抗体与SLE发生坏疽有关P值分别为0．012、0．004、0．037及0．087，OR值分别为1．03（95％CI1．01～1．05）、39．05（95％CI3．31～460．13）、17．99（95％CI1．19～271．29）及7．01（95％CI0．75～65．12）]。15例坏疽患者接受≥1mg·kg^-1·d^-1泼尼松治疗，18例接受环磷酰胺治疗，8例患者截肢。坏疽发生3周内积极给予≥1mg·kg^-1·d^-1泼尼松治疗能降低坏疽截肢风险P=0．081，OR=0．135（95％CI0．01—1．28）]。结论长病程、雷诺现象、C反应蛋白升高及抗SSA抗体是SLE合并坏疽的危险因素，早期积极治疗能够阻止坏疽进展、改善预后。

Clinical characteristics, risk factors and prognosis of systemic lupus erythematosus complicated with digital gangrene: a study of 2684 cases

Objective To study clinical manifestations, risk factors and prognosis of patients with systemic lupus erythematosus (SLE) complicated with digital gangrene. Methods Data of 2684 inpatients with SLE admired to Peking Union Medical College Hospital during December 1997 to August 2007, including demographic information, clinical features, laboratory findings, as well as therapeutic regimens, were retrospectively analyzed, and 18 of those complicated with digital gangrene were identified and followed up. Results Average age at onset in patients of SLE complicated with digital gangrene was(33±12)years, with an average duration of disease course of (99±60 ) months. Longer course duration ( ≥ 4 yrs), Raynaud's phenomenon, elevated serum C-reactive protein (CRP) and positive anti-SSA antibody all were associated with occurrence of digital gangrene in patients of SLE, with P values of 0.012, 0.004, 0.037 and 0.087 respectively, and odds ratios of 1.03 (95% CI 1.01～1.05 ), 39.05 (95% CI 3.31～460.13 ), 17.99 (95%(CI 1.19～271.29) and 7.01 (95% CI 0.75～65.12), respectively. Fifteen of 18 SLE patients complicated with digital gangrene started treatment with predisone ≥1 mg˙kg-1 ˙d-1, and eighteen all were treated with cyclophosphamide, though eight cases failed and ultimately received digital amputation. Prompt corticosteroid treatment (predisone ≥ 1 mg˙kg-1˙d-1 within three weeks after occurrence) could decrease risk for amputation, with P =0.081 and OR =0.135 (95% CI 0.01～1.28). Conclusions Longer duration of disease course, Raynaud's phenomenon, elevated serum CRP and positive anti-SSA antibody all are independent predictors of SLE complicated with digital gangrene. Early and aggressive treatment can prevent occurrence of gangrene from its progression and improve its prognosis.