Liver Transplantation for Primary Biliary Cirrhosis

Tzakis, Andreas G.; Carcassonne, Christian; Todo, Satoru; Makowka, Leonard; Starzl, Thomas E.

doi:10.1055/s-2008-1040506

Cited by 20 publications

(5 citation statements)

References 15 publications

(7 reference statements)

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“…Liver transplantation has greatly improved the survival of patients with PBC [9]. It is the only effective treatment for those with decompensated cirrhosis or liver failure.…”

Section: Liver Transplantationmentioning

confidence: 99%

See 1 more Smart Citation

Primary biliary cirrhosis: A 2010 update

Poupon

2010

Journal of Hepatology

248

206

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Primary biliary cirrhosis (PBC) is a chronic inflammatory autoimmune disease that mainly targets the cholangiocytes of the interlobular bile ducts in the liver. The condition primarily affects middle-aged women. Without treatment, PBC generally progresses to cirrhosis and eventually liver failure over a period of 10-20 years. PBC is a rare disease with prevalence of less than 1/2000. PBC is thought to result from a combination of multiple genetic factors and superimposed environmental triggers. The contribution of the genetic predisposition is evidenced by the familial clustering. Several risk factors, including exposure to infectious agents and chemical xenobiotics, have been suggested. Ursodeoxycholic acid (UDCA) is currently the only FDA-approved medical treatment for PBC. When administered at doses of 13-15 mg/kg/day, a majority of patients with PBC have a normal life expectancy without additional therapeutic measures. One out of three patients does not adequately respond to UDCA therapy and may need additional medical therapy and/or liver transplantation. This review summarises current knowledge on the epidemiology, ethiopathogenesis, clinical, and therapeutic aspects of PBC.

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“…Liver transplantation has greatly improved the survival of patients with PBC [9]. It is the only effective treatment for those with decompensated cirrhosis or liver failure.…”

Section: Liver Transplantationmentioning

confidence: 99%

“…Although the aetiologies and aetiopathogenesis are still elusive, most patients benefit from liver transplantation, a procedure routinely performed since 1989 [9], and ursodeoxycholic acid, a medical therapy now used worldwide for early stage disease [10].…”

Section: Introductionmentioning

confidence: 99%

Primary biliary cirrhosis: A 2010 update

Poupon

2010

Journal of Hepatology

248

206

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“…However, complications like Dupuytren's contractures from cirrhosis are usually irreversible [1]. In spite of the fact that the dermatological manifestations of liver disease such as autoimmune hepatitis, chronic cholestatic liver disease, and hepatitis C after LT have not been well documented, nevertheless studies propose that most of these conditions like alopecia and vitiligo associated with autoimmune hepatitis [11–13], xanthomas and xanthelasmas associated with chronic cholestatic liver disease [14], cryoglobulinemia, porphyria cutanea tarda, leukocytoclastic vasculitis, and lichen planus associated with hepatitis C [1, 15], and Kayser-Fleischer rings (KF rings) associated with Wilson's disease [1] improve significantly; however, KF rings are not considered as dermatological disorder.…”

Section: Preexisting Dermatological Conditions After Ltmentioning

confidence: 99%

Dermatological Disorders following Liver Transplantation: An Update

Yadav

Bai

Liang

2019

Canadian Journal of Gastroenterology and Hepatology

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Patients undergoing liver transplantation (LT) are at a high risk of dermatological complications compared to the general population as a result of long-term use of immunosuppressant. However, the risk is not as high as other solid organ transplantations (SOT), particularly for skin cancer. The liver is considered as an immune privileged organ since it has a low prevalence of humoral rejection in contrast to other SOT, and thus, LT requires a minimal amount of immunosuppressants compared to other SOT recipients. However, because of the large volume of the liver, patients with LT have higher donor lymphocytes that sometimes may trigger graft-versus-host-disease, yet it is rare. On the other hand, the vast majority of the nonspecific dermatological lesions linked with cirrhosis improve after removal of diseased liver or due to the immunosuppressant used after LT. Nevertheless, dermatological infections related to bacteria, viruses, and fungus after LT are not uncommon. Additionally, the incidence of IgE-mediated food allergies develops in 12.2% of LT patients and may present as life-threatening conditions such as urticaria and/or angioedema and hypersensitivity. Moreover, skin malignancies after LT are a matter of concern. Thus, posttransplant dermatological care should be provided to all LT patients for any suspicious dermatological lesions. Our goal is to give an outline of the dermatological manifestation associated with LT for the clinicians by collecting the published data from all archived case reports.

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“…68 Transplantation may be indicated also in case of bilirubin >6 mg/dL, untreatable pruritus, severe osteoporosis, inappropriate quality of life. 37 PBC relapses in about 20% of patients at 5 years.…”

Section: Liver Transplantationmentioning

confidence: 99%

Autoimmune liver diseases: internist’s guide from bench to bedside

Visconti¹

2015

Ital J Med

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Liver Transplantation for Primary Biliary Cirrhosis

Cited by 20 publications

References 15 publications

Primary biliary cirrhosis: A 2010 update

Primary biliary cirrhosis: A 2010 update

Dermatological Disorders following Liver Transplantation: An Update

Autoimmune liver diseases: internist’s guide from bench to bedside

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