Lacrimal Gland IgG4-Related Disease

**Semantic type:** Disease or Syndrome

**Definition:** A mass-forming lesion that affects the lacrimal gland and is characterized by the presence of a lymphoplasmacytic infiltrate rich in IgG4-positive plasma cells and storiform tissue fibrosis, and increased serum IgG4 levels. It responds to steroids treatment and follows a course of remissions and recurrences.