Bezafibrate for the treatment of primary sclerosing cholangitis

Mizuno, Suguru; Hirano, Keisuke; Tada, Minoru; Yamamoto, Keisuke; Yashima, Yoko; Yagioka, Hiroshi; Kawakubo, Kazumichi; Ito, Yukiko; Kogure, Hirofumi; Sasaki, Takashi; Arizumi, Toshihiko; Togawa, Osamu; Matsubara, Saburo; Sasahira, Naoki; Tsujino, Takeshi; Isayama, Hiroyuki; Kawabe, Takao; Omata, Masao; Koike, Kazuhiko

doi:10.1007/s00535-010-0204-x

Cited by 46 publications

(40 citation statements)

References 19 publications

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“…Notably, PPARαagonists had a negative effect in the Mdr2 –/– model, while genetic knockout of PPARα (by crossing Pparα – /– with Mdr2 –/– mice) cured the phenotype [34]. However, this could represent species-specific effects and preliminary clinical data suggest that – similar to reports in PBC – fibrates may have beneficial effects in PSC [37]. …”

Section: Treatmentmentioning

confidence: 77%

Primary Sclerosing Cholangitis: New Approaches to Diagnosis, Surveillance and Treatment

Trauner

Halilbasic²,

Baghdasaryan³

et al. 2012

Dig Dis

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Primary sclerosing cholangitis (PSC) is a chronic inflammatory bile duct disease of unknown etiology, frequently associated with inflammatory bowel disease and leading to end-stage liver disease requiring liver transplantation. Moreover, PSC is a premalignant condition associated with an increased risk for hepatobiliary and colorectal malignancy. Since effective medical therapy for PSC is still lacking, this disorder represents a potentially fatal disease with poor prognosis. This article is a summary of an overview given at the 5th Falk Gastro Conference in Munich 2012 and reviews the challenges associated with diagnosis, surveillance and therapy of PSC.

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Section: Treatmentmentioning

confidence: 77%

Primary Sclerosing Cholangitis: New Approaches to Diagnosis, Surveillance and Treatment

Trauner

Halilbasic²,

Baghdasaryan³

et al. 2012

Dig Dis

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“…Although this issue remains controversial, liver biopsy should not be contraindicated, at least for some patients, because the differential diagnosis between PSC and hepatic amyloidosis is important in deciding treatment policy, especially during the early stage of the disease. High‐dose chemotherapy in combination with hematopoietic stem cell transplantation for AL‐type amyloidosis has recently been started, with encouraging results in patients with good performance status and limited cardiac involvement, whereas liver‐protecting drugs such as ursodeoxycholic acid and bezafibrate are prescribed during early‐stage PSC …”

Section: Discussionmentioning

confidence: 99%

Two cases with hepatic amyloidosis suspected of having primary sclerosing cholangitis

Hirano

Ikemura

Mizuno

et al. 2013

Hepatology Research

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Sclerogenic biliary changes in hepatic amyloidosis are seldom observed. Here, we report two recent cases initially suspected as primary sclerosing cholangitis (PSC), which were later diagnosed as hepatic amyloidosis (AL type). Case 1: On the basis of magnetic resonance cholangiopancreatography (MRCP) findings, PSC was suspected in a 41-year-old woman with jaundice. Computed tomography (CT) showed nodular pulmonary lesions and swollen cervical, mediastinal and para-aortic lymph nodes, the cause of which was unknown despite detailed examinations. Because of rapid deterioration in the patient's liver function, living donor liver transplantation was performed. She was then diagnosed with hepatic amyloidosis, but died of heart failure due to cardiac amyloidosis 74 days after surgery. Case 2: On the basis of MRCP findings, PSC was suspected in a 49-year-old woman with jaundice. CT showed multiple cystic pulmonary lesions, and hypogammaglobulinemia was also observed (immunoglobulin G, 481 mg/dL). After a biliary plastic stent was placed, liver and lung biopsy confirmed the presence of amyloid deposition. These two cases indicate that it is important to consider hepatic amyloidosis as a differential diagnosis of PSC. The presence of atypical extrahepatic lesions may be useful clues for confirming the diagnosis.

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“…Bezafibrate has been successfully used as a pharmacological alternative in the PSC in a few cases reported in the literature, so that there is insufficient evidence to recommend its use (88,89) . Corticosteroids and other immunosuppressive agents are not recommended for the treatment of PSC, except in cases of PSC with features resembling AIH, the so called "AIH/ PSC overlap syndrome" (68) In this context, the combined regimen with prednisone and azathioprine is indicated, typically in association with UDCA.…”

Section: Pharmacological Treatment Of Pscmentioning

confidence: 99%

Brazilian society of hepatology recommendations for the diagnosis and management of autoimmune diseases of the liver

Bittencourt

Cançado

Couto

et al. 2015

Arq. Gastroenterol.

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-In order to draw evidence-based recommendations concerning the management of autoimmune diseases of the liver, the Brazilian Society of Hepatology has sponsored a single-topic meeting in October 18th, 2014 at São Paulo. An organizing committee comprised of seven investigators was previously elected by the Governing Board to organize the scientific agenda as well as to select twenty panelists to make a systematic review of the literature and to present topics related to the diagnosis and treatment of autoimmune hepatitis, primary sclerosing cholangitis, primary biliary cirrhosis and their overlap syndromes. After the meeting, all panelists gathered together for the discussion of the topics and the elaboration of those recommendations. The text was subsequently submitted for suggestions and approval of all members of the Brazilian Society of Hepatology through its homepage. The present paper is the final version of the reviewed manuscript organized in topics, followed by the recommendations of the Brazilian Society of Hepatology.

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Bezafibrate for the treatment of primary sclerosing cholangitis

Abstract: This study showed that bezafibrate could lower the levels of hepatobiliary enzymes in about half of a cohort of patients with PSC.

Cited by 46 publications

References 19 publications

Primary Sclerosing Cholangitis: New Approaches to Diagnosis, Surveillance and Treatment

Primary Sclerosing Cholangitis: New Approaches to Diagnosis, Surveillance and Treatment

Two cases with hepatic amyloidosis suspected of having primary sclerosing cholangitis

Brazilian society of hepatology recommendations for the diagnosis and management of autoimmune diseases of the liver

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