Overview of the Immunoglobulin G4-related Disease Spectrum.
10.15279/kpba.2015.20.3.124
- Author:
Sung Hoon MOON
1
;
Tae Jun SONG
;
Myung Hwan KIM
Author Information
1. Department of Internal Medicine, Hallym University Sacred Heart Hospital, Hallym University College of Medicine, Anyang, Korea.
- Publication Type:Review
- Keywords:
Immunoglobulin G4-related disease;
Autoimmune pancreatitis;
Autoimmune diseases
- MeSH:
Autoimmune Diseases;
Dacryocystitis;
Diagnosis;
Fibrosis;
Granuloma, Plasma Cell;
Immunoglobulin G;
Immunoglobulins*;
Kidney Diseases;
Mikulicz' Disease;
Pancreatitis;
Retroperitoneal Fibrosis;
Sialadenitis;
Thyroid Gland;
Thyroiditis
- From:Korean Journal of Pancreas and Biliary Tract
2015;20(3):124-129
- CountryRepublic of Korea
- Language:English
-
Abstract:
Immunoglobulin G4 (IgG4)-related disease is a newly named fibroinflammatory condition characterized by tumefactive lesions that contain dense lymphoplasmacytic infiltrates rich in IgG4-positive cells. Elevated serum IgG4 concentrations also often accompany IgG4-related disease. Many medical conditions that were long viewed as diseases confined to single organs (e.g., Mikulicz disease, type 1 autoimmune pancreatitis, Riedel's thyroiditis, multifocal fibrosclerosis, inflammatory pseudotumor, mediastinal fibrosis, retroperitoneal fibrosis, and etc.) are now designated as part of the spectrum of IgG4-related disease. The preferred nomenclature suggested by a committee of international experts uses the prefix "IgG4-related-" for individual organ involvement, regardless of the organ system affected. One exception is type 1 autoimmune pancreatitis (IgG4-related pancreatitis). Comprehensive diagnostic criteria for IgG4-related disease and organ-specific diagnostic criteria (e.g., IgG4-related dacryoadenitis and sialadenitis, type 1 autoimmune pancreatitis, IgG4-related kidney disease and IgG4-related sclerosing cholangitis) can aid clinicians in the diagnosis of this erratic condition.
