title: Organ Correlation in IgG4-Related Diseases.
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.
authors.affiliation: Department of Internal Medicine, Tokyo Metropolitan Komagome Hospital, Tokyo Japan.
authors.forename: Satomi
authors.identifier: 0000-0002-3840-1542
authors.initials: S
authors.lastname: Koizumi
authors.affiliation: Department of Internal Medicine, Tokyo Metropolitan Komagome Hospital, Tokyo Japan.
authors.forename: Terumi
authors.identifier: 0000-0002-2237-2767
authors.initials: T
authors.lastname: Kamisawa
authors.affiliation: Department of Internal Medicine, Tokyo Metropolitan Komagome Hospital, Tokyo Japan.
authors.forename: Sawako
authors.identifier: 0000-0002-4113-2718
authors.initials: S
authors.lastname: Kuruma
authors.affiliation: Department of Internal Medicine, Tokyo Metropolitan Komagome Hospital, Tokyo Japan.
authors.forename: Taku
authors.identifier: 0000-0003-1207-1087
authors.initials: T
authors.lastname: Tabata
authors.affiliation: Department of Internal Medicine, Tokyo Metropolitan Komagome Hospital, Tokyo Japan.
authors.forename: Kazuro
authors.identifier: 0000-0003-0324-859X
authors.initials: K
authors.lastname: Chiba
authors.affiliation: Department of Internal Medicine, Tokyo Metropolitan Komagome Hospital, Tokyo Japan.
authors.forename: Susumu
authors.identifier: 0000-0001-8390-6032
authors.initials: S
authors.lastname: Iwasaki
authors.affiliation: Department of Internal Medicine, Tokyo Metropolitan Komagome Hospital, Tokyo Japan.
authors.forename: Go
authors.identifier: 0000-0003-2368-230X
authors.initials: G
authors.lastname: Kuwata
authors.affiliation: Department of Internal Medicine, Tokyo Metropolitan Komagome Hospital, Tokyo Japan.
authors.forename: Takashi
authors.identifier: 0000-0002-6790-8713
authors.initials: T
authors.lastname: Fujiwara
authors.affiliation: Department of Internal Medicine, Tokyo Metropolitan Komagome Hospital, Tokyo Japan.
authors.forename: Junko
authors.identifier: 0000-0001-5820-1137
authors.initials: J
authors.lastname: Fujiwara
authors.affiliation: Department of Internal Medicine, Tokyo Metropolitan Komagome Hospital, Tokyo Japan.
authors.forename: Takeo
authors.identifier: 0000-0002-3739-0386
authors.initials: T
authors.lastname: Arakawa
authors.affiliation: Department of Internal Medicine, Tokyo Metropolitan Komagome Hospital, Tokyo Japan.
authors.forename: Koichi
authors.identifier: 0000-0002-6441-5841
authors.initials: K
authors.lastname: Koizumi
authors.affiliation: Department of Internal Medicine, Tokyo Metropolitan Komagome Hospital, Tokyo Japan.
authors.forename: Kumiko
authors.identifier: 0000-0002-4697-1780
authors.initials: K
authors.lastname: Momma
mesh_terms: D000328:Adult; D000368:Aged; D001327:Autoimmune Diseases / Q000453:epidemiology* / Q000276:immunology*; D015897:Comorbidity; D005260:Female; D006801:Humans; D007074:Immunoglobulin G / Q000276:immunology*; D007564:Japan / Q000453:epidemiology; D008297:Male; D008875:Middle Aged; D009102:Multiple Organ Failure / Q000453:epidemiology* / Q000276:immunology*; D009928:Organ Specificity / Q000276:immunology; D015995:Prevalence; D012307:Risk Factors; D013223:Statistics as Topic
chemical_list: D007074:Immunoglobulin G
keywords: Cholangitis, Sclerosing; IgG4; Pancreatitis