Recurrent Nodular Regenerative Hyperplasia Following Liver Transplantation in Common Variable Immunodeficiency

Hercun, Julian; Parikh, Esha; Kleiner, David E.; Fuss, Ivan J.; Uzel, Gülbû; Strober, Warren; Koh, Christopher; Holland, Steven M.; Heller, Theo

doi:10.1002/hep.31775

Cited by 8 publications

(9 citation statements)

References 4 publications

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“…However, recurrence of PSVD after LT had not been well studied. 7,10,49 In the largest cohort described up to now, including 16 patients transplanted with PSVD, recurrence was observed in 2 cases (13%). Furthermore, in a systematic review including 26 patients, a single case of recurrence, 7 y after LT, was observed.…”

Section: Discussionmentioning

confidence: 99%

“…PSVD recurrence was definitively confirmed by liver biopsy in only one of these patients; interestingly, this patient had a CVID, and post-LT PSVD recurrence has been previously reported in several patients with CVID. 49 Considering the worst scenario, in case these 4 patients would have had recurrent PSVD, this would be a recurrence rate of 5.1%. This is still a figure similar or even smaller to the recurrence rate described in other liver conditions such as primary biliary cholangitis or primary sclerosing cholangitis after a similar follow-up time.…”

Section: Discussionmentioning

confidence: 99%

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Liver Transplantation for Porto-sinusoidal Vascular Liver Disorder: Long-term Outcome

et al. 2023

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Background. Porto-sinusoidal vascular liver disorder (PSVD) is a rare disease that occasionally requires liver transplantation (LT), despite usually presenting preserved liver function. There remains a paucity of data pertaining to LT in PSVD. The aim was to identify features associated with post-LT outcomes in PSVD. Methods. Retrospective multicentre study of 79 patients who received LT for PSVD. Results. Median post-LT follow-up was 37 (range 1-261) mo. Refractory ascites 24 (30%), hepatic encephalopathy 16 (20%), and hepatopulmonary syndrome 13 (16.3%) were the most frequent indications for LT. Hepatocellular carcinoma was the indication in only 2 patients. Twenty-four patients died, 7 due to liver and 17 to non-liver related causes. Post-LT survival was 82.2%, 80.7%, and 68.6% at 1, 2, and 5 y, respectively. Post-LT survival was significantly better in patients without (n = 58) than in those with a persistent severe PSVD-associated condition (n = 21). Pre-LT hyperbilirubinemia levels and creatinine >100 µmol/L were also independently associated with poor survival. Six patients (7.6%) required a second LT. Recurrence of PSVD was confirmed by liver biopsy in only 1 patient and in 3 further patients it was likely. Conclusions. LT in PSVD is associated with an acceptable outcome in the absence of associated severe conditions. However, persistence of a severe associated condition, pre-LT high bilirubin levels, or creatinine >100 µmol/L impact outcome, and these are features that should be considered when evaluating PSVD patients for LT. PSVD recurrence is possible after LT and needs to be explored, at least, in cases of posttransplant portal hypertension.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Liver Transplantation for Porto-sinusoidal Vascular Liver Disorder: Long-term Outcome

et al. 2023

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“…Patients should also be monitored for porto-pulmonary hypertension [ 13 ] or hepatopulmonary syndrome, as described in immune-deficiency disorders such as common-variable immunodeficiency. Often, development of hepatopulmonary syndrome leads to liver transplantation, although NRH may recur after transplant [ 14 ].…”

Section: Discussionmentioning

confidence: 99%

Nodular Regenerative Hyperplasia of the liver in Juvenile Dermatomyositis

et al. 2022

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Background We present two cases of Nodular Regenerative Hyperplasia (NRH) associated with Juvenile Dermatomyositis (JDM). Case Presentation Case 1: A nine-year-old Caucasian male with refractory JDM and anti-NXP2 autoantibodies was diagnosed at age two. Over seven years, he developed arthritis, dysphagia, dysphonia, severe calcinosis, and colitis. Complications included recurrent cellulitis, infections, and hepatosplenomegaly. Multiple medications were chronically used, including prednisone, methotrexate, azathioprine, cyclophosphamide, mycophenolate mofetil, rituximab, tacrolimus, etanercept, abatacept, infliximab, and tocilizumab. Case 2: A 19-year-old Asian female with chronically active JDM and anti-MDA5 autoantibodies was diagnosed at age 15. Symptomatology included ulcerative skin lesions, Raynaud’s phenomenon with digital ulcers, arthritis, interstitial lung disease with pulmonary hypertension, and calcinosis. Medications included chronic use of prednisone, methotrexate, abatacept, cyclophosphamide, mycophenolate mofetil, rituximab, tofacitinib, and sildenafil. In both patients, clinical symptomatology was not suggestive of liver disease or portal hypertension, but laboratory studies revealed elevated serum transaminases with progressive thrombocytopenia and no active liver-associated infections. The first patient’s liver ultrasound showed coarse hepatic texture with mild echogenicity, splenomegaly, and portal hypertension. The second patient’s liver ultrasound was normal, but elastography indicated increased stiffness. Liver biopsy confirmed NRH in both patients. Conclusions It is difficult to recognize NRH in JDM, as it often presents with elevated transaminases which may be mistaken for JDM muscle flare, corticosteroid-related fatty liver, or medication-related transaminitis. NRH has been associated with several medications used to treat JDM, including methotrexate, azathioprine, and cyclophosphamide, which should be discontinued if NRH develops. Providers should consider NRH in JDM patients with severe, refractory disease who have persistently elevated transaminases and persistent thrombocytopenia.

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“…NRH‐associated liver disease is also linked in case reports to Hepatopulmonary syndrome with intrapulmonary arteriovenous shunting and may cause dual organ failure. [ 74–76 ] Figure 5A,B shows histopathologic evidence of NRH on liver biopsy of our patient with CVID. AIH‐like liver disease is also distinct from classic AIH, with occurrence of the inflammatory infiltrates, interface hepatitis ,and piecemeal necrosis superimposed on nodular liver parenchyma, and no autoantibodies given the inability to synthesize them.…”

Section: Clinical Approach To Liver Disease In Ieismentioning

confidence: 94%

“…[ 12 ] Splenomegaly, ascites, and esophageal varices are seen in patients with NRH patients with noncirrhotic portal hypertension. [ 74 ] NRH can contribute greatly to morbidity and mortality in patients with CVID. NRH‐associated liver disease is also linked in case reports to Hepatopulmonary syndrome with intrapulmonary arteriovenous shunting and may cause dual organ failure.…”

Section: Clinical Approach To Liver Disease In Ieismentioning

confidence: 99%

Tip of the iceberg: A comprehensive review of liver disease in Inborn errors of immunity

et al. 2022

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Inborn errors of immunity (IEIs) consist of numerous rare, inherited defects of the immune system that affect about 500,000 people in the United States.As advancements in diagnosis through genetic testing and treatment with targeted immunotherapy and bone marrow transplant emerge, increasing numbers of patients survive into adulthood posing fresh clinical challenges.A large spectrum of hepatobiliary diseases now present in those with immunodeficiency diseases, leading to morbidity and mortality in this population. Awareness of these hepatobiliary diseases has lagged the improved management of the underlying disorders, leading to missed opportunities to improve clinical outcomes. This review article provides a detailed description of specific liver diseases occurring in various inborn errors of immunity.A generalized approach to diagnosis and management of hepatic complications is provided, and collaboration with hepatologists, immunologists, and pathologists is emphasized as a requirement for optimizing management and outcomes.

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Recurrent Nodular Regenerative Hyperplasia Following Liver Transplantation in Common Variable Immunodeficiency

Cited by 8 publications

References 4 publications

Liver Transplantation for Porto-sinusoidal Vascular Liver Disorder: Long-term Outcome

Liver Transplantation for Porto-sinusoidal Vascular Liver Disorder: Long-term Outcome

Nodular Regenerative Hyperplasia of the liver in Juvenile Dermatomyositis

Tip of the iceberg: A comprehensive review of liver disease in Inborn errors of immunity

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