Abstract

• General pathway of bile acid formation and primary associated cholestatic liver diseases (CLD) are described. • Primary biliary cholangitis and primary sclerosis cholangitis along with their pathogenesis, symptoms and diagnosis have been discussed. • Current advances in both allopathic and natural treatments of PBC and PSC included. • Genetic approaches for PBC and PSC discussed. Cholestatic liver diseases (CLD) are one of the hepatic disorders that lead to scarring of hepatic tissues due to abnormalities like obstruction of bile flow, hepatitis and alcohol abuse. The primary biliary cholangitis (PBC) and primary sclerosis cholangitis (PSC) are slow progressive autoimmune diseases, which are the foremost types of CLD. The pathogenesis for the disease is unclear till date. Only limited therapies are available and liver transplant is the final resort for the same but the number of patients waiting for liver transplantation is quite higher than the number of active donors. Moreover, it is also associated with high risk also like rejection of donated liver and in a study, about 25% of mortality rate within five years of transplantation has been reported. There are some recent advancements in allopathic and natural treatment and some drugs under clinical trials are preferred only to prolong the timeline for liver transplantation but do not treat the disease completely. This review mentions about the pathogenicity, treatments including natural compounds and drugs in use as well as under clinical trials. The future RNA and DNA approaches that can be explored to treat CLD and result in an effective approach for the prevention of this disease have also been discussed here. We are confident that the current review study will assist future medicinal researchers in their quest of rational approaches for the treatment of primary biliary cholangitis and primary sclerosis cholangitis.

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