Systematic review: recurrent autoimmune liver diseases after liver transplantation

Montano‐Loza, Aldo J.; Bhanji, Rahima A.; Wasilenko, Shawn; Mason, Andrew L.

doi:10.1111/apt.13894

Cited by 103 publications

(108 citation statements)

References 148 publications

(206 reference statements)

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“…The only existing life‐saving treatment option is liver transplantation. Nevertheless, PSC reoccurs in 20%‐37% of patients after liver transplantation . No drugs have been shown to be effective in preventing death, liver transplantation or cholangiocarcinoma (CCA).…”

Section: Primary Sclerosing Cholangitismentioning

confidence: 99%

The future of autoimmune liver diseases – Understanding pathogenesis and improving morbidity and mortality

Engel

Taubert

Jaeckel

et al. 2020

Liver International

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Autoimmune liver diseases (AILD), namely autoimmune hepatitis (AIH), primary biliary cholangitis (PBC) and primary sclerosing cholangitis (PSC), are rare diseases. These days, patients with PBC almost never require liver transplantation. When treated early with ursodeoxycholic acid patients have a normal life expectancy if the disease is diagnosed at an early stage and the patients respond to treatment. Patients with AIH often go into remission with first‐line therapy including corticosteroids alone or in combination with azathioprine. Nevertheless, about one quarter of patients already developed cirrhosis at diagnosis. Those who do not respond to first line standard of care (SOC) have significant liver‐related morbidity and mortality. No approved second‐ or third‐line treatments are available and the drugs are selected based on limited case series and personal experience. Larger trials are needed to develop efficient therapies for difficult‐to‐treat AIH patients. No treatment has been found to alter the natural course of disease in patients with PSC except for liver transplantation. Identifying PSC patients at risk of developing cholangiocarcinoma (CCA) is another unmet need. Current research in all AILD including AIH, PBC and PSC, focuses on improving our understanding of the underlying disease process and identifying new therapeutic targets to decrease morbidity and mortality.

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Section: Primary Sclerosing Cholangitismentioning

confidence: 99%

The future of autoimmune liver diseases – Understanding pathogenesis and improving morbidity and mortality

Engel

Taubert

Jaeckel

et al. 2020

Liver International

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“…Elevated liver enzymes and immunoglobulins before LT and lymphoplasmacytic infiltration with moderate‐to‐severe inflammatory activity in explants may be associated with a greater likelihood of AIH recurrence after LT. Complete corticosteroid withdrawal should be dependent on these factors …”

Section: Timing Of Ltmentioning

confidence: 99%

Acute Severe Autoimmune Hepatitis: Corticosteroids or Liver Transplantation?

et al. 2019

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Acute severe presentations of autoimmune hepatitis (AIH) represent a challenge for the transplant community. As a disease, it is poorly characterized, and there is a weak evidence base to guide diagnosis and treatment. Early identification of acute severe AIH is key because it determines the initiation of corticosteroids, which can be lifesaving. However, their use in this setting remains controversial. The Model for End‐Stage Liver Disease score, severity of coagulopathy, and grade of encephalopathy may be predictors of outcome with corticosteroid therapy. The optimal timing of liver transplantation (LT) can be difficult to determine and, as such, the decision to proceed to transplantation should not be delayed by protracted courses of corticosteroids. The aim of this review is to better characterize this subset of patients; to differentiate them clinically, serologically, and histologically from chronic AIH and other causes of acute liver failure; and to present the role, predictors, and optimal timings of corticosteroid therapy and LT. Although this review is specific to adults, many principles hold true for the pediatric population.

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“…The diagnosis of recurrence is challenging because the features may overlap with other posttransplant complications. Risk factors for recurrence have been reported to include young donor age, an older recipient, a long cold ischemia time, and specific immunosuppressive drugs (tacrolimus has been associated with increased risk, and cyclosporine with decreased risk) . Although treatment of recurrent PBC with UDCA has been effective in improving liver biochemistry, it remains controversial whether the use of UDCA prevents recurrence.…”

Section: Viral Hepatitismentioning

confidence: 99%

Longterm outcome of the liver graft: A clinician's perspective—recurrent disease, the universal shifting

Strasser

2017

Liver Transpl

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Systematic review: recurrent autoimmune liver diseases after liver transplantation

Cited by 103 publications

References 148 publications

The future of autoimmune liver diseases – Understanding pathogenesis and improving morbidity and mortality

The future of autoimmune liver diseases – Understanding pathogenesis and improving morbidity and mortality

Acute Severe Autoimmune Hepatitis: Corticosteroids or Liver Transplantation?

Longterm outcome of the liver graft: A clinician's perspective—recurrent disease, the universal shifting

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