Current approach to the diagnosis of IgG4-related disease - Combination of comprehensive diagnostic and organ-specific criteria.
IgG4-related disease (IgG4-RD) is a fascinating clinical entity proposed by Japanese investigators, and includes a wide variety of diseases, formerly diagnosed as Mikulicz's disease (MD), autoimmune pancreatitis (AIP), interstitial nephritis, prostatitis, retroperitoneal fibrosis, etc. Although all clinicians in every field of medicine may encounter this new disease, a unifying diagnostic criterion has not been established. In 2011, the Japanese IgG4 team, organized by the Ministry of Health, Labor and Welfare (MHLW) of Japan, published comprehensive diagnostic criteria for IgG4-RD. Several problems with these criteria have arisen in clinical practice, however, including the difficulty obtaining biopsy samples from some patients, and the sensitivity and the specificity of techniques used to measure serum IgG4 concentrations. Although serum IgG4 concentration is an important clinical marker for IgG4-RD, its diagnostic utility in differentiating IgG4-RD from other diseases, called IgG4-RD mimickers, remains unclear. This review describes the current optimal approach for the diagnosis of IgG4-RD, based on both comprehensive and organ-specific diagnostic criteria, in patients with diseases such as IgG4-related pancreatitis (AIP), sclerosing cholangitis, and renal, lung and orbital diseases.
a Division of RA and Autoimmune Diseases , Internal Medicine, Nagahama City Hospital , Shiga , Japan.
Hisanori
H
Umehara
b The Third Department of Internal Medicine, Division of Gastroenterology and Hepatology , Kansai Medical University , Osaka , Japan.
Kazuichi
K
Okazaki
a Division of RA and Autoimmune Diseases , Internal Medicine, Nagahama City Hospital , Shiga , Japan.
Takuji
T
Nakamura
a Division of RA and Autoimmune Diseases , Internal Medicine, Nagahama City Hospital , Shiga , Japan.
Tomomi
T
Satoh-Nakamura
c Division of Rheumatology , Internal Medicine, Kudo General Hospital , Ishikawa , Japan.
Akio
A
Nakajima
d Division of Rheumatology, Department of Internal Medicine, Graduate School of Medical Science , Kanazawa University , Ishikawa , Japan.
Mitsuhiro
M
Kawano
e Department of Clinical Immunology, Graduate School of Medicine , Kyoto University , Kyoto , Japan.
Tsuneyo
T
Mimori
f Department of Gastroenterology and Hepatology, Graduate School of Medicine , Kyoto University , Kyoto , Japan.
Tsutomu
T
Chiba
D001327:Autoimmune Diseases / Q000097:blood / Q000175:diagnosis* / Q000276:immunology; D003937:Diagnosis, Differential; D006801:Humans; D007074:Immunoglobulin G / Q000097:blood* / Q000276:immunology; D007564:Japan
D007074:Immunoglobulin G
IgG4-RD; Mikulicz’s disease; autoimmune pancreatitis; diagnostic criteria; orbital disease; sclerosing cholangitis