Organ Correlation in IgG4-Related Diseases.
10.3346/jkms.2015.30.6.743
- Author:
Satomi KOIZUMI
1
;
Terumi KAMISAWA
;
Sawako KURUMA
;
Taku TABATA
;
Kazuro CHIBA
;
Susumu IWASAKI
;
Go KUWATA
;
Takashi FUJIWARA
;
Junko FUJIWARA
;
Takeo ARAKAWA
;
Koichi KOIZUMI
;
Kumiko MOMMA
Author Information
1. Department of Internal Medicine, Tokyo Metropolitan Komagome Hospital, Tokyo Japan. kamisawa@cick.jp
- Publication Type:Original Article
- Keywords:
IgG4;
Pancreatitis;
Cholangitis, Sclerosing
- MeSH:
Adult;
Aged;
Autoimmune Diseases/*epidemiology/*immunology;
Comorbidity;
Female;
Humans;
Immunoglobulin G/*immunology;
Japan/epidemiology;
Male;
Middle Aged;
Multiple Organ Failure/*epidemiology/*immunology;
Organ Specificity/immunology;
Prevalence;
Risk Factors;
Statistics as Topic
- From:Journal of Korean Medical Science
2015;30(6):743-748
- CountryRepublic of Korea
- Language:English
-
Abstract:
IgG4-related disease (IgG4-RD) is a potentially multiorgan disorder. In this study, clinical and serological features from 132 IgG4-RD patients were compared about organ correlations. Underlying pathologies comprised autoimmune pancreatitis (AIP) in 85 cases, IgG4-related sclerosing cholangitis (IgG4-SC) in 12, IgG4-related sialadenitis (IgG4-SIA) in 56, IgG4-related dacryoadenitis (IgG4-DAC) in 38, IgG4-related lymphadenopathy (IgG4-LYM) in 20, IgG4-related retroperitoneal fibrosis (IgG4-RF) in 19, IgG4-related kidney disease (IgG4-KD) in 6, IgG4-related pseudotumor (IgG4-PT) in 3. Sixty-five patients (49%) had multiple IgG4-RD (two affected organs in 36 patients, three in 19, four in 8, five in 1, and six in 1). Serum IgG4 levels were significantly higher with multiple lesions than with a single lesion (P<0.001). The proportion of association with other IgG4-RD was 42% in AIP, the lowest of all IgG4-RDs. Serum IgG4 level was lower in AIP than in other IgG4-RDs. Frequently associated IgG4-RDs were SIA (25%) and DAC (12%) for AIP; AIP (75%) for IgG4-SC; DAC (57%), AIP (38%) and LYM (27%) for IgG4-SIA; AIP (26%) and LYM (26%) for IgG4-DAC; SIA (75%), DAC (50%) and AIP (45%) for IgG4-LYM; SIA (58%), AIP (42%) and LYM (32%) for IgG4-RF; AIP (100%) and SIA (67%) for IgG4-KID; and DAC (67%) and SIA (67%) for IgG4-PT. Most associated IgG4-RD lesions were diagnosed simultaneously, but IgG4-SIA and IgG4-DAC were sometimes identified before other lesions. About half of IgG4-RD patients had multiple IgG4-RD lesions, and some associations were seen between specific organs.
