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2016 ; 64
(2
): 501-7
Nephropedia Template TP
gab.com Text
Twit Text FOAVip
Twit Text #
English Wikipedia
Immunoglobulin G4(+) B-cell receptor clones distinguish immunoglobulin G
4-related disease from primary sclerosing cholangitis and biliary/pancreatic
malignancies
#MMPMID27015613
Doorenspleet ME
; Hubers LM
; Culver EL
; Maillette de Buy Wenniger LJ
; Klarenbeek PL
; Chapman RW
; Baas F
; van de Graaf SF
; Verheij J
; van Gulik TM
; Barnes E
; Beuers U
; de Vries N
Hepatology
2016[Aug]; 64
(2
): 501-7
PMID27015613
show ga
Immunoglobulin G4 (IgG4)-related disease (IgG4-RD) of the biliary tree and
pancreas is difficult to distinguish from sclerosing cholangitis and
biliary/pancreatic malignancies (CA). An accurate noninvasive test for diagnosis
and monitoring of disease activity is lacking. We demonstrate that dominant
IgG4(+) B-cell receptor (BCR) clones determined by next-generation sequencing
accurately distinguish patients with IgG4-associated cholangitis/autoimmune
pancreatitis (n = 34) from those with primary sclerosing cholangitis (n = 17) and
CA (n = 17). A novel, more affordable, and widely applicable quantitative
polymerase chain reaction (qPCR) protocol analyzing the IgG4/IgG RNA ratio in
blood also achieves excellent diagnostic accuracy (n = 125). Moreover, this qPCR
test performed better than serum IgG4 levels in sensitivity (94% vs. 86%) and
specificity (99% vs. 73%) and correlates with treatment response (n = 20).
CONCLUSIONS: IgG4(+) BCR clones and IgG4/IgG RNA ratio markedly improve
delineation, early diagnosis, and monitoring of IgG4-RD of the biliary tree and
pancreas. (Hepatology 2016;64:501-507).