TY - JOUR
T1 - IgG4-associated cholangitis
AU - Beuers, Ulrich
AU - De Buy Wenniger, Lucas J.Maillette
AU - Doorenspleet, Marieke
AU - Hubers, Lowiek
AU - Verheij, Joanne
AU - Van Gulik, Thomas
AU - Van De Graaf, Stan F.J.
AU - De Vries, Niek
PY - 2014/7
Y1 - 2014/7
N2 - IgG4-associated cholangitis (IAC) is the hepatobiliary manifestation of immunoglobulin G4-related disease (IgG4-RD), a systemic fibroinflammatory disorder with a wide variety of clinical presentations and organ manifestations. IgG4-RD predominantly affects the hepatobiliary tract (IAC) and pancreas (autoimmune pancreatitis) and mimics hepatobiliary, pancreatic and other malignancies. Patients typically are 60-80 years old and 80-85% are male. They often present with painless obstructive jaundice and organ swelling that can be mistaken for pancreatic or bile duct cancer, as well as primary or secondary sclerosing cholangitis. An accurate diagnostic marker is lacking and extensive surgery for presumed malignant hepatobiliary or pancreatic disease leads to the diagnosis of IgG4-RD in 1 of 3 patients. Early effective immunosuppressive treatment is often missed. The pathogenesis of IgG4-RD has been enigmatic. We recently identified dominant IgG4+ B-cell receptor clones in blood and tissue of patients with IAC, but not in healthy or disease controls, and hypothesized that specific B-cell responses are pivotal to the pathogenesis of IAC and IgG4-RD. Analysis of our Amsterdam cohort and blinded extramural validation of the Oxford cohort of patients with IgG4-RD disclosed a remarkable association with 'blue-collar work'. Thus, long-term exposure to solvents and other organic agents might predispose to IgG4-RD.
AB - IgG4-associated cholangitis (IAC) is the hepatobiliary manifestation of immunoglobulin G4-related disease (IgG4-RD), a systemic fibroinflammatory disorder with a wide variety of clinical presentations and organ manifestations. IgG4-RD predominantly affects the hepatobiliary tract (IAC) and pancreas (autoimmune pancreatitis) and mimics hepatobiliary, pancreatic and other malignancies. Patients typically are 60-80 years old and 80-85% are male. They often present with painless obstructive jaundice and organ swelling that can be mistaken for pancreatic or bile duct cancer, as well as primary or secondary sclerosing cholangitis. An accurate diagnostic marker is lacking and extensive surgery for presumed malignant hepatobiliary or pancreatic disease leads to the diagnosis of IgG4-RD in 1 of 3 patients. Early effective immunosuppressive treatment is often missed. The pathogenesis of IgG4-RD has been enigmatic. We recently identified dominant IgG4+ B-cell receptor clones in blood and tissue of patients with IAC, but not in healthy or disease controls, and hypothesized that specific B-cell responses are pivotal to the pathogenesis of IAC and IgG4-RD. Analysis of our Amsterdam cohort and blinded extramural validation of the Oxford cohort of patients with IgG4-RD disclosed a remarkable association with 'blue-collar work'. Thus, long-term exposure to solvents and other organic agents might predispose to IgG4-RD.
KW - B-cell receptor clones
KW - IgG4-associated cholangitis
KW - IgG4-related disease
KW - Serum levels of IgG4
UR - http://www.scopus.com/inward/record.url?scp=84904622154&partnerID=8YFLogxK
U2 - https://doi.org/10.1159/000360513
DO - https://doi.org/10.1159/000360513
M3 - Article
C2 - 25034294
SN - 0257-2753
VL - 32
SP - 605
EP - 608
JO - Digestive Diseases
JF - Digestive Diseases
IS - 5
ER -