Pharmacological interventions for primary sclerosing cholangitis

Saffioti, Francesca; Gurusamy, Kurinchi Selvan; Hawkins, Neil; Toon, Clare D; Tsochatzis, Emmanuel; Davidson, Brian R; Thorburn, Douglas

doi:10.1002/14651858.cd011343.pub2

Cited by 21 publications

(16 citation statements)

References 115 publications

(21 reference statements)

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“…There are various treatments for SSC, 13 but the best way remains unclear. Among pharmacotherapies, UDCA and immunosuppressants have been used for primary sclerosing cholangitis and are thought to be useful in SSC 14 . In our case, ALP and GGT showed a poor response to reduced glutathione, polyene phosphatidylcholine and compound glycyrrhizin.…”

Section: Discussionmentioning

confidence: 61%

Case report: Secondary sclerosing cholangitis induced by lapatinib and vinorelbine in a metastasis breast cancer patient

Zhang

Qin

et al. 2021

Thoracic Cancer

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Secondary sclerosing cholangitis (SSC) is a rare cholestatic liver disease that may have a severe clinical course. A 61‐year‐old woman with a history of metastasis breast cancer was admitted to our hospital for the second cycle of chemotherapy with lapatinib and vinorelbine. The patient had no reports of elevated liver function tests (LFTs) in the previous multiple chemotherapies or history of liver disease. However, the admission laboratory results showed severe cholestatic liver injury with the possibility of SSC by magnetic resonance cholangiopancreatography. Although chemotherapy was discontinued and patient was treated with hepatoprotective drugs, the LFTs did not improve and liver biopsy indicated mild injury of intrahepatic bile duct epithelium and hepatocyte. We added ursodeoxycholic acid and prednisolone to protect the liver, and laboratory data showed a response. To prevent the progression, lapatinib and vinorelbine were reintroduced and transient increases in alanine aminotransferase and γ‐glutamyl transpeptidase were observed. With no evidence of viral or autoimmune liver disease, SSC induced by lapatinib and vinorelbine was diagnosed. This is the first case report of tyrosine kinase inhibitors and vinorelbine induced SSC and clinicians should be aware of the possibility of it. More case reports about this adverse drug reaction are needed to delineate optimal management.

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Section: Discussionmentioning

confidence: 61%

Case report: Secondary sclerosing cholangitis induced by lapatinib and vinorelbine in a metastasis breast cancer patient

Zhang

Qin

et al. 2021

Thoracic Cancer

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“…The treatment of PSC is largely an unmet need and currently includes medical and endoscopic measures, short of liver transplantation 110,111 . UDCA has been investigated in several clinical trials, with conflicting results.…”

Section: Therapymentioning

confidence: 99%

Rheumatic Manifestations in Autoimmune Liver Disease

Selmi

Generali

Gershwin

2018

Rheumatic Disease Clinics of North America

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Autoimmune liver diseases coexist with rheumatic disorders in approximately 30% of cases and may also share pathogenic mechanisms. Autoimmune liver diseases result from an immune-mediated injury of different tissues, with autoimmune hepatitis (AIH) targeting hepatocytes, and primary biliary cholangitis (PBC) and primary sclerosing cholangitis targeting cholangiocytes. Sjogren syndrome is diagnosed in 7% of AIH cases and serologic autoimmunity profiles are a common laboratory abnormality, particularly in the case of serum antimitochondrial (PBC) or anti-liver kidney microsomal antibodies (AIH). Therapeutic strategies may overlap between rheumatic and autoimmune liver diseases and practitioners should be vigilant in managing bone loss.

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“…That search only found few existing reviews, all of which focused narrowly on QoL in PSC. One of these, a Cochrane review of pharmacological interventions for PSC, included QoL as an outcome measure [ 20 ]; another explored the impact of itch on QoL for cholestatic liver disease [ 21 ]. There was therefore a lack of clarity about how QoL had been measured in this population, which factors were important determinants of QoL in PSC, and how the condition affected QoL.…”

Section: Introductionmentioning

confidence: 99%

Quality of life in primary sclerosing cholangitis: a systematic review

Marcus

Stone

Krooupa

et al. 2021

Health Qual Life Outcomes

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Background Primary sclerosing cholangitis (PSC) is a rare bile duct and liver disease which can considerably impact quality of life (QoL). As part of a project developing a measure of QoL for people with PSC, we conducted a systematic review with four review questions. The first of these questions overlaps with a recently published systematic review, so this paper reports on the last three of our initial four questions: (A) How does QoL in PSC compare with other groups?, (B) Which attributes/factors are associated with impaired QoL in PSC?, (C) Which interventions are effective in improving QoL in people with PSC?. Methods We systematically searched five databases from inception to 1 November 2020 and assessed the methodological quality of included studies using standard checklists. Results We identified 28 studies: 17 for (A), ten for (B), and nine for (C). Limited evidence was found for all review questions, with few studies included in each comparison, and small sample sizes. The limited evidence available indicated poorer QoL for people with PSC compared with healthy controls, but findings were mixed for comparisons with the general population. QoL outcomes in PSC were comparable to other chronic conditions. Itch, pain, jaundice, severity of inflammatory bowel disease, liver cirrhosis, and large-duct PSC were all associated with impaired QoL. No associations were found between QoL and PSC severity measured with surrogate markers of disease progression or one of three prognostic scoring systems. No interventions were found to improve QoL outcomes. Conclusion The limited findings from included studies suggest that markers of disease progression used in clinical trials may not reflect the experiences of people with PSC. This highlights the importance for clinical research studies to assess QoL alongside clinical and laboratory-based outcomes. A valid and responsive PSC-specific measure of QoL, to adequately capture all issues of importance to people with PSC, would therefore be helpful for clinical research studies.

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Pharmacological interventions for primary sclerosing cholangitis

Cited by 21 publications

References 115 publications

Case report: Secondary sclerosing cholangitis induced by lapatinib and vinorelbine in a metastasis breast cancer patient

Case report: Secondary sclerosing cholangitis induced by lapatinib and vinorelbine in a metastasis breast cancer patient

Rheumatic Manifestations in Autoimmune Liver Disease

Quality of life in primary sclerosing cholangitis: a systematic review

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