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2014 ; 95
(3
): 209-15
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Frequency and significance of IgG4 immunohistochemical staining in liver explants
from patients with primary sclerosing cholangitis
#MMPMID24750423
Fischer S
; Trivedi PJ
; Ward S
; Greig PD
; Therapondos G
; Hirschfield GM
Int J Exp Pathol
2014[Jun]; 95
(3
): 209-15
PMID24750423
show ga
Dense tissue infiltrates of IgG4(+) plasma cells >50/high-powered field (HPF) are
purportedly highly specific for IgG4-related disease. However, the frequency and
significance of liver-infiltrating IgG4(+) plasma cells in primary sclerosing
cholangitis (PSC) applying these cut-offs has not been determined. We sought to
determine the incidence of intrahepatic IgG4-positive staining in PSC patients
undergoing transplantation, correlating findings with clinical parameters.
Immunohistochemical staining was performed on liver explants obtained between
1991 and 2009. Of 122 explants obtained, hilar IgG4(+) staining was found to be
mild (10-29 IgG4(+) cells/HPF) in 23.0%, moderate (30-50/HPF) in 9.0% and marked
(>50/HPF) in 15.6%. Marked hilar lymphoplasmacytic infiltration was significantly
associated with marked hilar IgG4(+) staining (P < 0.001). No patient had marked
peripheral IgG4(+) staining, although mild and moderate staining was observed in
24.5% and 3.3% respectively. Marked hilar IgG4(+) staining was significantly
associated with the presence of dominant biliary strictures (P = 0.01) and need
for biliary stenting (P = 0.001). There did not, however, exist any significant
differences in the age at PSC diagnosis, presence of inflammatory bowel disease
or extrahepatic autoimmune disease, frequency of cholangiocarcinoma, interval
between diagnosis and transplantation, or post-transplant PSC recurrence or
survival. Of 51 control liver sections (PBC = 18; HCV = 19; HBV = 8; AIH = 6),
none had marked or moderate hilar IgG4(+) staining, whereas mild staining was
seen in only 10% (P < 0.001). Marked (>50/HPF) hilar IgG4(+) lymphoplasmacytic
infiltration is frequently observed in PSC and associated with the presence of
dominant biliary strictures. However, unlike serum IgG4(+) , this does not
seemingly associate with clinical disease course.