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2014 ; 13
(4-5
): 441-4
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The diagnosis of primary biliary cirrhosis
#MMPMID24424173
Bowlus CL
; Gershwin ME
Autoimmun Rev
2014[Apr]; 13
(4-5
): 441-4
PMID24424173
show ga
Primary biliary cirrhosis (PBC) is a chronic liver disease characterized by the
immune mediated destruction of small intrahepatic bile duct epithelial cells
leading to cholestasis and cirrhosis. The autoimmune basis of PBC is supported by
the highly specific anti-mitochondrial antibodies (AMAs) and autoreactive T
cells, the former being the basis for diagnosis in the vast majority of cases.
Although a rare disease, the incidence rates of PBC have been increasing,
possibly due to increased testing and diagnosis as opposed to a true increase in
disease incidence. Presently, most cases are asymptomatic and only suspected
based upon routine liver tests. Those with symptoms typically complain of
pruritus and fatigue. The diagnosis of PBC is based on the presence of at least 2
of 3 key criteria including a persistently elevated serum alkaline phosphatase,
the presence of serum AMAs, and liver histology consistent with PBC. Anti-nuclear
antibodies specific to PBC are useful in cases in which AMAs are not detected and
may indicate a more aggressive course. Ursodeoxycholic acid is the only proven
therapy for PBC and in most cases can delay or prevent disease progression.
However, a subgroup of patients does not adequately respond to ursodeoxycholic
acid and for whom new therapies are needed.
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