系统性红斑狼疮并发假性肠梗阻的临床特点

Systemic lupus erythematosus associated with intestinal pseudo-obstruction

目的通过分析系统性红斑狼疮(SLE)合并假性肠梗阻(IPO)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4例SLE合并IPO住院患者的临床及实验室特点等。结果4例SLE合并IPO患者,以IPO为首发2例,并同时有肾盂输尿管积水,膀胱壁增厚;胆囊壁水肿1例、抗核抗体阳性4例、血清狼疮细胞阳性4例、P-ANCA阳性3例、抗ds-DNA抗体阳性1例、抗SM抗体阳性1例。4例患者均给与大量糖皮质激素治疗,其中2例辅以环磷酰胺(CTX)治疗,2例反应良好、1例反应慢、1例反应差。结论IPO是SLE的一种少见但严重的并发症,常合并肾盂输尿管积水、膀胱壁增厚、胆囊壁增厚,提示由内脏平滑肌受累运动障碍所致;尽早诊断,及时使用大量糖皮质激素或合用免疫抑制剂治疗,对于控制病情,改善预后至关重要,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）. Methods With review of the literature, 4 cases of SLE with IPO were analyzed retrospectively. Results Two cases manifested initially with IPO and had concurrent bilateral ureterohydronephrosis and thickened bladder wall; 1 had hydrocholeeystis. All cases were ANA and LE cell positive; 3 cases were P-ANCA positive; 1 anti-dsDNA positive and 1 anti-SM positive. All patients were administered to high dose steroid therapy, two of whom were added to immunodepressants. Two cases responded well to the therapy, 1 improved slowly and 1 had the bad clinic effect. Conclusions IPO is the rare but severe complication of SLE. 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.