CAJ | 학술논문

目的：分析狼疮肠系膜血管炎（LMV）患者的临床资料，提高临床医生对 LMV 的认识。方法收集2010年6月-2013年9月上海仁济医院风湿科确诊的28例 LMV 患者，回顾性分析其临床表现、合并系统损害情况、实验室检查结果、治疗及预后。结果（1）主要临床表现：腹泻22例（78.6%），腹痛21例（75.0%），恶心和/或呕吐19例（67.9%），发热11例（39.3%）。（2）合并系统损害情况：单纯消化道受累4例（14.3%），合并1个及以上其他系统的损害24例（85.7%），其中血液系统受累16例（57.1%），14例行心脏超声检查患者中心包积液8例（57.1%），肾脏受累13例（46.4%），皮肤黏膜受累9例（32.1%），输尿管肾盂积水8例（28.6%），16例行腹部立卧位 X 线检查患者中假性肠梗阻（IPO）4例（25.0%），呼吸系统受累4例（14.3%）。（3）16例（57.1%）患者红细胞沉降率（ESR）升高。24例患者行血脂检查，其中66.7%（16/24）载脂蛋白 A（Apo - A）水平异常（14例 Apo- A 水平降低，2例 Apo - A 水平升高），37.5%（9/24）高密度脂蛋白（HDL）水平异常（8例 HDL 水平降低，1例HDL 水平升高）。补体 C3水平下降24例（85.7%），补体 C4水平下降14例（50.0%）。（4）4例合并 IPO 患者中3例抗 SSA 抗体阳性；8例合并输尿管肾盂积水患者中6例抗 SSA 抗体阳性；（5）23例患者行腹部 CT 检查，其中肠壁增厚水肿15例（65.2%）；（6）均给予糖皮质激素治疗，其中26例（92.9%）患者给予中到大剂量激素治疗后有效，症状缓解，2例（7.1%）患者并发重症感染死亡。结论 LMV 临床表现以腹痛、腹泻、呕吐为主，常与其他系统损害并存，多处于疾病活动；大部分患者伴 ESR 增快、血脂异常、补体 C3及 C4下降；抗 SSA 抗体可能与 IPO 及输尿管肾盂积水有关；腹部增强 CT 检查有助于早期诊断；多数 LMV 患者对大剂量激素治疗的反应效果好。目的：分析狼瘡腸繫膜血管炎（LMV）患者的臨床資料，提高臨床醫生對 LMV 的認識。方法收集2010年6月-2013年9月上海仁濟醫院風濕科確診的28例 LMV 患者，迴顧性分析其臨床錶現、閤併繫統損害情況、實驗室檢查結果、治療及預後。結果（1）主要臨床錶現：腹瀉22例（78.6%），腹痛21例（75.0%），噁心和/或嘔吐19例（67.9%），髮熱11例（39.3%）。（2）閤併繫統損害情況：單純消化道受纍4例（14.3%），閤併1箇及以上其他繫統的損害24例（85.7%），其中血液繫統受纍16例（57.1%），14例行心髒超聲檢查患者中心包積液8例（57.1%），腎髒受纍13例（46.4%），皮膚黏膜受纍9例（32.1%），輸尿管腎盂積水8例（28.6%），16例行腹部立臥位 X 線檢查患者中假性腸梗阻（IPO）4例（25.0%），呼吸繫統受纍4例（14.3%）。（3）16例（57.1%）患者紅細胞沉降率（ESR）升高。24例患者行血脂檢查，其中66.7%（16/24）載脂蛋白 A（Apo - A）水平異常（14例 Apo- A 水平降低，2例 Apo - A 水平升高），37.5%（9/24）高密度脂蛋白（HDL）水平異常（8例 HDL 水平降低，1例HDL 水平升高）。補體 C3水平下降24例（85.7%），補體 C4水平下降14例（50.0%）。（4）4例閤併 IPO 患者中3例抗 SSA 抗體暘性；8例閤併輸尿管腎盂積水患者中6例抗 SSA 抗體暘性；（5）23例患者行腹部 CT 檢查，其中腸壁增厚水腫15例（65.2%）；（6）均給予糖皮質激素治療，其中26例（92.9%）患者給予中到大劑量激素治療後有效，癥狀緩解，2例（7.1%）患者併髮重癥感染死亡。結論 LMV 臨床錶現以腹痛、腹瀉、嘔吐為主，常與其他繫統損害併存，多處于疾病活動；大部分患者伴 ESR 增快、血脂異常、補體 C3及 C4下降；抗 SSA 抗體可能與 IPO 及輸尿管腎盂積水有關；腹部增彊 CT 檢查有助于早期診斷；多數 LMV 患者對大劑量激素治療的反應效果好。
목적：분석랑창장계막혈관염（LMV）환자적림상자료，제고림상의생대 LMV 적인식。방법수집2010년6월-2013년9월상해인제의원풍습과학진적28례 LMV 환자，회고성분석기림상표현、합병계통손해정황、실험실검사결과、치료급예후。결과（1）주요림상표현：복사22례（78.6%），복통21례（75.0%），악심화/혹구토19례（67.9%），발열11례（39.3%）。（2）합병계통손해정황：단순소화도수루4례（14.3%），합병1개급이상기타계통적손해24례（85.7%），기중혈액계통수루16례（57.1%），14례행심장초성검사환자중심포적액8례（57.1%），신장수루13례（46.4%），피부점막수루9례（32.1%），수뇨관신우적수8례（28.6%），16례행복부립와위 X 선검사환자중가성장경조（IPO）4례（25.0%），호흡계통수루4례（14.3%）。（3）16례（57.1%）환자홍세포침강솔（ESR）승고。24례환자행혈지검사，기중66.7%（16/24）재지단백 A（Apo - A）수평이상（14례 Apo- A 수평강저，2례 Apo - A 수평승고），37.5%（9/24）고밀도지단백（HDL）수평이상（8례 HDL 수평강저，1례HDL 수평승고）。보체 C3수평하강24례（85.7%），보체 C4수평하강14례（50.0%）。（4）4례합병 IPO 환자중3례항 SSA 항체양성；8례합병수뇨관신우적수환자중6례항 SSA 항체양성；（5）23례환자행복부 CT 검사，기중장벽증후수종15례（65.2%）；（6）균급여당피질격소치료，기중26례（92.9%）환자급여중도대제량격소치료후유효，증상완해，2례（7.1%）환자병발중증감염사망。결론 LMV 림상표현이복통、복사、구토위주，상여기타계통손해병존，다처우질병활동；대부분환자반 ESR 증쾌、혈지이상、보체 C3급 C4하강；항 SSA 항체가능여 IPO 급수뇨관신우적수유관；복부증강 CT 검사유조우조기진단；다수 LMV 환자대대제량격소치료적반응효과호。
Objective To identify the clinical features of lupus mesenteric vasculitis(LMV)and to enhance the un-derstanding of LMV among clinical doctors. Methods A total of 28 patients who were diagnosed with LMV in department of rheumatism,Shanghai Renji Hospital,from June 2010 to September 2013,were selected as study subjects,the clinical mani-festations,other system organ lesions,laboratory test results,treatment and prognosis were retrospectively reviewed. Results Main clinical manifestations:22(78. 6% )cases had diarrhea,21(75. 0% )cases had abdominal pain,19(67. 9% )cases had nausea and/ or vomiting,11(39. 3% )cases had fever. Other system organ lesions:4(14. 3% )cases had simple arch-enteric lesions,24(85. 7% )cases had multiple system involvement(≥1 system),16(57. 1% )cases had blood system le-sions,among 14 cases who received cardiac ultrasound,8(57. 1% )cases had pericardial effusion,13(46. 4% )cases had renal lesions,9(32. 1% )cases had skin and mucosa lesions,8(28. 6% )cases had hydroureter and hydronephrosis,among 16 cases who performed abdomen film on erect and supine positions,4 (25. 0% )cases had intestinal pseudo obstruction (IPO),4(14. 3% )cases had respiratory system lesions. Increased level of ESR existed in 16(57. 1% ) cases,among 24 cases who received blood lipid test,abnormal Apo - A level existed in 16(66. 7% )cases(low Apo - A level existed in 14, high Apo - A level existed in 2),abnormal HDL level existed in 9(37. 5% )cases(low HDL level existed in 8,high HDL level existed in 1). Decreased level of complement C3 existed in 24(85. 7% )cases,and decreased level of complement C4 ex-isted in 14(50. 0% )cases. Among 4 cases who had both LMV and IPO,3 cases were anti - SSA positive. Besides,among 8 cases who had both LMV and hydronephrosis,6 cases were anti - SSA positive. Among 23 cases who received abdominal CT ex-amination,15(65. 2% )cases had bowel wall thickening and edema. All cases received corticosteroid treatment,symptoms improved in 26 patients(92. 9% )after treatment with median to large dosage of corticosteroid,however the other 2 patients (7. 1% )died from severe infection. Conclusion The clinical characteristics of LMV includes abdominal pain,diarrhea,vom-iting;LMV frequently coexists with other system damages in active SLE patients. Increased level of ESR,dyslipidemia,de-creased level of complement C3 and C4 existed in most patients. Enhanced abdominal CT examination is helpful for early diagnosis of LMV,and anti - SSA antibody may be related to IPO and hydronephrosis. The curative effect of large dosage corticosteroid in treatment of most LMV patients is good.