IgG4-Related Disease with Emphasis on Its Gastrointestinal Manifestation.

IgG4-related disease is an immune-mediated fibroinflammatory condition with a diverse spectrum of organ involvement, commonly in the pancreas and bile ducts among other organs such as salivary and lacrimal glands. Classic histopathologic findings are the gold standard for confirmation of diagnosis, although diagnosis remains challenging, as biomarkers to date are neither sufficient nor necessary. Glucocorticoids are the most effective initial treatment, generally having a dramatic response, although limited clinical evidence exists regarding effective maintenance therapy. This review summarizes key GI manifestations of this condition for the practicing gastroenterologist and addresses the pathology, disease mechanism, and current therapeutic recommendations.