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2015 ; 30
(6
): 743-8
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Organ Correlation in IgG4-Related Diseases
#MMPMID26028927
Koizumi S
; Kamisawa T
; Kuruma S
; Tabata T
; Chiba K
; Iwasaki S
; Kuwata G
; Fujiwara T
; Fujiwara J
; Arakawa T
; Koizumi K
; Momma K
J Korean Med Sci
2015[Jun]; 30
(6
): 743-8
PMID26028927
show ga
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.