Multifocal gastrointestinal varices: a rare manifestation of immunoglobulin G4-related disease

Multifocal gastrointestinal varices are uncommon and often associated with liver cirrhosis. They consist of varices at the gastroesophageal region and the other sites (i.e. ectopic varices) simultaneously. The etiology includes venous system anomalies or thrombosis (congenital or acquired), vascular injury (iatrogenic or traumatic), or portal hypertension (either intrahepatic or extrahepatic). The clinical manifestations vary from asymptomatic lesions to life-threatening variceal hemorrhage. The identification of bleeding foci, as well as the etiology of varices, can be challenging. The treatment necessitates a multidisciplinary approach. Here, we report a case with multifocal gastrointestinal varices involving the stomach, duodenum, and transverse colon. The patient presented with intermittent melena and has no history of liver or heart disease. Serial endoscopic examinations confirmed the multiple sites of the gastrointestinal varices. Abdominal computed tomography demonstrated that the liver parenchyma is normal, and the hepatic veins, intrahepatic portal system, as well as vena cava, are all unobstructed. Nevertheless, it revealed typical features of autoimmune pancreatitis, retroperitoneal fibrosis, and compromised splenic and superior mesenteric veins. After the pancreatic tissue sampling, we eventually confirmed the etiology as immunoglobulin G4-related disease. In addition to steroid treatment for immunoglobulin G4-related disease, we successfully treated variceal bleeding with band ligation and prevented rebleeding with propranolol. He had been convalescing and has received periodic follow-up in our outpatient clinic for more than 12 months uneventfully.

多灶性胃肠道静脉曲张（multifocal gastrointestinal varices）较为少见，且常与肝硬化相关。此类病变指同时累及胃食管区域及其他部位（即异位静脉曲张（ectopic varices））的静脉曲张。其病因涵盖静脉系统异常、血栓形成（先天性或获得性）、血管损伤（医源性或创伤性），或门静脉高压（肝内型或肝外型）。临床表现差异显著，可从无症状病变进展为危及生命的静脉曲张出血。准确识别出血灶及明确静脉曲张病因均存在一定挑战，治疗需采用多学科诊疗模式。本次我们报告1例累及胃、十二指肠及横结肠的多灶性胃肠道静脉曲张病例。患者表现为间歇性黑便，无肝脏或心脏疾病史。系列内镜检查证实其存在多部位胃肠道静脉曲张。腹部计算机断层扫描结果显示，肝实质正常，肝静脉、肝内门静脉系统及腔静脉均通畅，但可见典型的自身免疫性胰腺炎、腹膜后纤维化表现，以及脾静脉和肠系膜上静脉受累。经胰腺组织活检后，最终明确病因是免疫球蛋白G4相关疾病（immunoglobulin G4-related disease）。在针对该免疫球蛋白G4相关疾病予以类固醇治疗的同时，我们采用套扎术成功控制静脉曲张出血，并予普萘洛尔预防再出血。患者目前已进入康复阶段，已在我院门诊接受为期12个月以上的定期随访，期间病情平稳，未出现异常情况。