PBC is a chronic liver condition resulting from progressive destruction of the intrahepatic bile duct and eventually leading to cirrhosis.
Knowing that PBC might have an autoimmune etiology, why isn't it treated with immunosuppressive therapy (like: corticosteroids, azathioprine.. etc)?
I think the pathogenesis of PBC is not understood well enough at this point. See Article Primary Biliary Cirrhosis
for a nice review of the subject. Some immunosuppressives that have been evaluate in PBC were somewhat beneficial but usually at the cost of high toxicity.
If the ursodexicolic works well and control with efficacy the progression of the disease it is not necessary to use
In some cases it is possible to use theses drugs. But if UDCA works, it is not necessary to use, because they have adverse effects. In addition, there is another choice for nonresponders - obeticholic acid.
It is used only in few cases. The ours therapy is enough effective and free of side effects
Thank you for your insightful answers. What I want to understand is: why isn't PBC treated as an autoimmune disease despite its autoimmune etiology?
I do know that it's treated with UDCA and that's the most effective treatment we have so far, but I want to know why.
As it is mentioned the mainstay of the PBC treatment is UDCA and other drugs in that category like obeticholic acid. Many other issues like diet and vitamin supplement, bone health etc should be carefully monitored and treated accordingly.
For your information I should say that there has been studies using corticosteroids and budesonide in the treatment of PBC. the ersult have not been convincing.
(Leuschner M, Maier KP, Schlichting J, et al. Oral budesonide and ursodeoxycholic acid for treatment of primary biliary cirrhosis: results of a prospective double-blind trial. Gastroenterology 1999; 117:918.
Rautiainen H, Kärkkäinen P, Karvonen AL, et al. Budesonide combined with UDCA to improve liver histology in primary biliary cirrhosis: a three-year randomized trial. Hepatology 2005; 41:747.)
But ongoing trials are being done in Europe on co-therapy of UDCA and budesonide too
We were working on a case of Primary Biliary Cholangitis recently.
The clinical set up , the biochemical markers , immunological markers & Liver biopsy work up many a times does not qualify for the the expected score for a definitive diagnosis as recommended by international hepatitis group.
Ref: International Autoimmune Hepatitis Group * Report: Review of criteria for diagnosis of autoimmune hepatitis. Journal of Hepatology 1999,31:929-938
The habitual treatment is UDCA that is effective and safer than any immunosuppressive drug
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