IgG4-related sclerosing oesophagitis in a 9-year-old girl

Abstract

Immunoglobulin G4 (IgG4)-related disease is a fibroinflammatory disorder characterised by a histopathological pattern of dense lymphoplasmocytic inflammation, fibrosis, obliterative phlebitis, and demonstration of dominance of IgG4-positive plasmocytes. Recognition of the disease entity is vital due its frequent misdiagnosis as a malignant tumour, which can result in unnecessary treatment measures including surgery. Despite recent insights into the mechanisms behind IgG4-related disease, a review of the literature yields only 13 (adult) cases of IgG4-related disease occuring within the oesophagus.

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