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2015 ; 94
(15
): e680
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IgG4-related disease: dataset of 235 consecutive patients
#MMPMID25881845
Inoue D
; Yoshida K
; Yoneda N
; Ozaki K
; Matsubara T
; Nagai K
; Okumura K
; Toshima F
; Toyama J
; Minami T
; Matsui O
; Gabata T
; Zen Y
Medicine (Baltimore)
2015[Apr]; 94
(15
): e680
PMID25881845
show ga
Immunoglobulin G4-related disease (IgG4-RD) is a recently discovered systemic
condition, in which various organ manifestations are linked by a similar
histological appearance. Our knowledge of this condition is still fragmented, as
most studies have examined only a few dozen patients or focused on a particular
organ manifestation. This study was conducted to learn the demography and patient
characteristics of IgG4-RD using a large cohort. A total of 235 consecutive
patients with IgG4-RD, diagnosed in 8 general hospitals in the same medical
district, were identified by searching the institutions' radiology database.
Inclusion criteria were histology-proven IgG4-RD according to the Pathology
Consensus Statement and/or definitive type 1 autoimmune pancreatitis meeting the
International Consensus Diagnostic Criteria. Clinical notes and images of
selected patients were retrospectively reviewed. All patients were adults
(M/F?=?4/1). The median age was 67 years (range 35-86). Nine tenths were
diagnosed in their 50s to 70s. Among 486 manifestations identified in total, the
most common was pancreatitis diagnosed in 142 patients (60%), followed by
sialadenitis (34%), tubulointerstitial nephritis (23%), dacryoadenitis (23%), and
periaortitis (20%). The majority of patients (95%) had at least 1 of the 5 most
common manifestations. Male and female patients differed in their organ
manifestations (periaortitis more common in males and sialodacryoadenitis more
common in females). Serum IgG4 (normal ?135?mg/dL) was elevated to >135?mg/dL in
208 patients (88%) and >270?mg/dL in 167 (71%). The IgG4 value was significantly
higher in patients with multiorgan involvement than in those with a single
manifestation (median 629?mg/dL vs 299?mg/dL, P?10% in 194 (89%). Corticosteroids were effective, but the relapse rate was
estimated to be 24% in the study period (median 37 months). During the follow-up,
15 malignant diseases were diagnosed in 13 patients (6%). This figure is similar
to the incidence (12.9 cancers) expected from the Japanese nationwide study for
cancer epidemiology (standardized incidence ratio 1.16). In conclusion, this
reliable dataset could improve the characterization of IgG4-RD, particularly its
unique demography and the frequency of each organ manifestation.
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