系统性红斑狼疮并发假性肠梗阻及输尿管肾盂积水的临床及实验室特点

Intestinal pseudo-obstruction and ureterohydronephrosis in systemic lupus erythematosus

目的通过分析系统性红斑狼疮(SLE)并发假性肠梗阻(IPO)及输尿管肾盂积水患者的临床及实验室特点,以提高临床医生对此并发症的认识。方法回顾性分析本院2000—2005年SLE并发IPO患者10例,收集临床及实验室资料,分析其发病情况、病情活动情况及受累脏器、病程、治疗及预后特点。结果以肠梗阻为首发者3例,同时伴输尿管肾盂积水者7例(7/9),膀胱壁增厚、毛糙5例(5/9),伴有胆囊壁增厚或壁毛糙者6例(6/8)。抗核抗体阳性10例,抗dsDNA抗体阳性6例,抗SSA抗体阳性6例,抗核糖核蛋白(RNP)抗体阳性5例,抗心磷脂抗体IgG阳性4例(4/7)。所有病例均有血清补体C3和C4的降低。肠梗阻症状均在大剂量糖皮质激素治疗1周左右缓解。结论IPO常与输尿管肾盂积水及胆囊壁增厚相伴发,提示由内脏平滑肌受累本身运动障碍所致,及时使用肾上腺糖皮质激素治疗对于控制病情、改善预后非常重要,应避免不必要的手术干预。

Objective To enhance the understanding of systemic lupus erythematosus （SLE） associated with intestinal pseudo-obstruction （IPO） and ureterohydronephrosis. Methods Ten cases of SLE with IPO and ureterohydronephrosis were analyzed retrospectively. Results IPO was the initial manifestation of SLE in three patients. Seven cases （7/9） had concurrent bilateral ureterohydronephrosis, 5 （5/9） had thickened bladder wall, and 6 cases （6/8） were associated with thickened gallbladder wall simultaneously. All cases were ANA positive and hypoeomplementemia, 6 had positive anti-dsDNA, 5 had positive anti-SSA, 6 had positive anti-RNP, and 4 were ACA-IgG positive （4/7）. IPO responded readily to high dose steroid therapy within one week in all patients. Conclusions The pathogenic mechanism of IPO is not fully understood. The coexistence of IPO, ureterohydronephrosis and thickened gallbladder wall probably suggests a visceral smooth muscle dysmotility. Early recognition of the syndrome is necessary for the initiation of the appropriate medical treatment and prevention of inappropriate surgical intervention. Hydronephrosis