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2017 ; 23
(14
): 2459-2469
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Pathogenesis and clinical spectrum of primary sclerosing cholangitis
#MMPMID28465630
Gidwaney NG
; Pawa S
; Das KM
World J Gastroenterol
2017[Apr]; 23
(14
): 2459-2469
PMID28465630
show ga
Primary sclerosing cholangitis (PSC) is a disease of the biliary tract, which has
been documented in the literature since 1867. This disease has a strong
predilection for affecting men and can be seen in individuals as young as 2 years
of age. PSC has a strong associated with inflammatory bowel disease, more
commonly with ulcerative colitis, and is also part of the clinical spectrum of
IgG4-related diseases. Small-duct PSC, a variant of PSC, also has an association
with inflammatory bowel disease. The exact pathogenesis of PSC is not well
understood at present, however, is likely a combination of a genetic
predisposition with alteration of the molecular structure of the gut. Abnormal
serum liver chemistry and presence of certain autoimmune markers are usually the
first indicators leading to a diagnosis of PCS, however, these may often be
normal in early stages of this disease. The diagnosis is made by cholangiography,
which is now considered the gold standard. PSC is a known pre-malignant
condition. Such patients have an increased risk of developing cholangiocarcinoma,
gallbladder neoplasia, and colon cancer. Many new treatment modalities have
emerged in the recent past, including anti-tumor necrosis factor- ? and
anti-integrins; however, liver transplantation is the only known cure for PSC.
Despite past and present research, PSC remains an enigmatic biliary disease with
few viable treatment options.