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

ABSTRACT

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.

KEYWORDS:

• Gastrointestinal hemorrhages
• autoimmune diseases
• endoscopy
• gastrointestinal
• collateral circulation
• gastric varices

Acknowledgments

The authors would like to thank all the colleagues who contributed to this study, especially the highly professional radiologist, Yi-Sheng Liu (Department of Diagnostic Radiology, National Cheng Kung University Hospital), and the experienced pathologists (Department of Pathology, National Cheng Kung University Hospital).

Compliance with Ethical Standards

The research was conducted according to the principles of the Declaration of Helsinki, and the informed consent was obtained from the patient.

Declaration of interest

The authors have no other relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript. This includes employment, consultancies, honoraria, stock ownership or options, expert testimony, grants or patents received or pending, or royalties. English editing was utilized on this manuscript, and it was provided by Wallace Academic Editing. Peer reviewers on this manuscript have no relevant financial relationships to disclose.

Author contributions

Tien-Ching Lin and Wei-Lun Chang collected the data and wrote the manuscript.

Supplementary material

Supplemental data for this article can be accessed here.

Additional information

Funding

This manuscript was not funded. This research received no specific grant from any funding agency in the public, commercial, or nonprofit sectors.