IgG4-related diseases

Evidence: Antibody

Names and Subtypes

Eosinophilic angiocentric fibrosis
Idiopathic hypocomplementemic tubulointerstitial nephritis
Inflammatory aortic aneurysm
IgG4-related disease
IgG4-related systemic disease
Inflammatory pseudotumor
Küttner’s tumor
Mediastinal fibrosis
Mikulicz’s syndrome
Multifocal fibrosclerosis
Ormond’s disease
Periaortitis
Periarteritis
Retroperitoneal fibrosis
Riedel’s thyroiditis

Description

IgG4 is a type of antibody normally found in humans. However, in some persons, the level of this antibody is elevated and it plays a role in the development of several autoimmune diseases, including myasthenia gravis, pemphigus, autoimmune thrombocytopenic purpura and autoimmune pancreatitis.

IgG4-related diseases (IgG4-RD) are complex fibro-inflammatory disorder that can affect any organ. Medical practitioners assign a diagnosis based on symptoms and the organ affected. The primary clinical feature in IgG4-RD entails a tumor-like presentation coupled with tissue-destructive lesions.

For more information, see the pages on specific diseases. At this time, not all IgG4 diseases are documented, and many are very rare.