Management of liver disease and portal hypertension in common variable immunodeficiency (CVID)

Ameratunga, 2013, New diagnostic criteria for common variable immune deficiency (CVID), which may assist with decisions to treat with intravenous or subcutaneous immunoglobulin, Clin Exp Immunol, 174, 203, 10.1111/cei.12178 Roskin, 2015, IgH sequences in common variable immune deficiency reveal altered B cell development and selection, 7, 302ra135 Notarangelo, 2009, International Union of Immunological Societies Expert Committee on Primary I. Primary immunodeficiencies: 2009 update, J Allergy Clin Immunol, 124, 1161, 10.1016/j.jaci.2009.10.013 Pecoraro, 2020, Heterogeneity of liver disease in common variable immunodeficiency disorders, Front Immunol, 11, 338, 10.3389/fimmu.2020.00338 Modell, 2018, Global report on primary immunodeficiencies: 2018 update from the Jeffrey Modell Centers Network on disease classification, regional trends, treatment modalities, and physician reported outcomes, Immunol Res, 66, 367, 10.1007/s12026-018-8996-5 Yazdani, 2020, Common variable immunodeficiency: epidemiology, pathogenesis, clinical manifestations, diagnosis, classification, and management, J Investig Allergol Clin Immunol, 30, 14, 10.18176/jiaci.0388 Hammarstrom, 2000, Selective IgA deficiency (SIgAD) and common variable immunodeficiency (CVID), Clin Exp Immunol, 120, 225, 10.1046/j.1365-2249.2000.01131.x Boyle, 2007, Population prevalence of diagnosed primary immunodeficiency diseases in the United States, J Clin Immunol, 27, 497, 10.1007/s10875-007-9103-1 Bonilla, 2005, Practice parameter for the diagnosis and management of primary immunodeficiency, Ann Allergy Asthma Immunol, 94, S1, 10.1016/S1081-1206(10)61142-8 Salzer, 2012, Common variable immunodeficiency: an update, Arthritis Res Ther, 14, 223, 10.1186/ar4032 Zietkiewicz, 2020, Shorter diagnostic delay in polish adult patients with common variable immunodeficiency and symptom onset after 1999, Front Immunol, 11, 982, 10.3389/fimmu.2020.00982 Oksenhendler, 2008, Infections in 252 patients with common variable immunodeficiency, Clin Infect Dis, 46, 1547, 10.1086/587669 Cunningham-Rundles, 1999, Common variable immunodeficiency: clinical and immunological features of 248 patients, Clin Immunol, 92, 34, 10.1006/clim.1999.4725 Resnick, 2012, Morbidity and mortality in common variable immune deficiency over 4 decades, Blood, 119, 1650, 10.1182/blood-2011-09-377945 Marschall, 2015, The Swiss national registry for primary immunodeficiencies: report on the first 6 years’ activity from 2008 to 2014, Clin Exp Immunol, 182, 45, 10.1111/cei.12661 Yazdani, 2017, Infectious and noninfectious pulmonary complications in patients with primary immunodeficiency disorders, J Investig Allergol Clin Immunol, 27, 213, 10.18176/jiaci.0166 Salavoura, 2008, Development of cancer in patients with primary immunodeficiencies, Anticancer Res, 28, 1263 Sarmiento, 2005, [Autoimmune disease in primary antibody deficiencies], Allergol Immunopathol (Madr), 33, 69, 10.1157/13072916 Uzzan, 2016, Gastrointestinal disorders associated with common variable immune deficiency (CVID) and chronic granulomatous disease (CGD), Curr Gastroenterol Rep, 18, 17, 10.1007/s11894-016-0491-3 Song, 2018, Common variable immunodeficiency and liver involvement, Clin Rev Allergy Immunol, 55, 340, 10.1007/s12016-017-8638-z Graziano, 2017, Delay in diagnosis affects the clinical outcome in a cohort of cvid patients with marked reduction of iga serum levels, Clin Immunol, 180, 1, 10.1016/j.clim.2017.03.011 Bonilla, 2016, International consensus document (ICON): common variable immunodeficiency disorders, J Allergy Clin Immunol Pract, 4, 38, 10.1016/j.jaip.2015.07.025 Ho, 2020, Non-infectious complications of common variable immunodeficiency: updated clinical spectrum, sequelae, and insights to pathogenesis, Front Immunol, 11, 149, 10.3389/fimmu.2020.00149 Wehr, 2015, Multicenter experience in hematopoietic stem cell transplantation for serious complications of common variable immunodeficiency, J Allergy Clin Immunol, 135, 988, 10.1016/j.jaci.2014.11.029 Milito, 2019, Double-blind, placebo-controlled, randomized trial on low-dose azithromycin prophylaxis in patients with primary antibody deficiencies, J Allergy Clin Immunol, 144, 584, 10.1016/j.jaci.2019.01.051 Cunningham-Rundles, 2008, Autoimmune manifestations in common variable immunodeficiency, J Clin Immunol, 28, S42, 10.1007/s10875-008-9182-7 Lima, 2022, Liver disease accompanied by enteropathy in common variable immunodeficiency: common pathophysiological mechanisms, Front Immunol, 13, 933463, 10.3389/fimmu.2022.933463 Farmer, 2017, Common variable immunodeficiency non-infectious disease endotypes redefined using unbiased network clustering in large electronic datasets, Front Immunol, 8, 1740, 10.3389/fimmu.2017.01740 Malamut, 2008, Nodular regenerative hyperplasia: the main liver disease in patients with primary hypogammaglobulinemia and hepatic abnormalities, J Hepatol, 48, 74, 10.1016/j.jhep.2007.08.011 Ward, 2008, Abnormal liver function in common variable immunodeficiency disorders due to nodular regenerative hyperplasia, Clin Exp Immunol, 153, 331, 10.1111/j.1365-2249.2008.03711.x Fuss, 2013, Nodular regenerative hyperplasia in common variable immunodeficiency, J Clin Immunol, 33, 748, 10.1007/s10875-013-9873-6 Gioia, 2018, Natural history of patients with non cirrhotic portal hypertension: comparison with patients with compensated cirrhosis, Dig Liver Dis, 50, 839, 10.1016/j.dld.2018.01.132 Siramolpiwat, 2014, Idiopathic portal hypertension: natural history and long-term outcome, Hepatology, 59, 2276, 10.1002/hep.26904 Azzu, 2019, Liver disease is common in patients with common variable immunodeficiency and predicts mortality in the presence of cirrhosis or portal hypertension, J Allergy Clin Immunol Pract, 7, 2484, 10.1016/j.jaip.2019.04.016 De Gottardi, 2019, Porto-sinusoidal vascular disease: proposal and description of a novel entity, Lancet Gastroenterol Hepatol, 4, 399, 10.1016/S2468-1253(19)30047-0 Wanless, 1990, Micronodular transformation (nodular regenerative hyperplasia) of the liver: a report of 64 cases among 2,500 autopsies and a new classification of benign hepatocellular nodules, Hepatology, 11, 787, 10.1002/hep.1840110512 Schouten, 2015, Idiopathic non-cirrhotic portal hypertension: a review, Orphanet J Rare Dis, 10, 67, 10.1186/s13023-015-0288-8 Valla, 2018, Vascular liver diseases on the clinical side: definitions and diagnosis, new concepts, Virchows Arch, 473, 3, 10.1007/s00428-018-2331-3 Reshamwala, 2006, Nodular regenerative hyperplasia: not all nodules are created equal, Hepatology, 44, 7, 10.1002/hep.21258 Jharap, 2015, Diagnosing nodular regenerative hyperplasia of the liver is thwarted by low interobserver agreement, PLoS One, 10, 10.1371/journal.pone.0120299 Kleiner, 2015, Noncirrhotic portal hypertension: pathology and nomenclature, Clin Liver Dis (Hoboken), 5, 123, 10.1002/cld.459 Bakshi, 2020, Nodular regenerative hyperplasia - an under-recognized vascular disorder of liver, Pathol Res Pract, 216, 152833, 10.1016/j.prp.2020.152833 Cazals-Hatem, 2011, Obliterative portal venopathy: portal hypertension is not always present at diagnosis, J Hepatol, 54, 455, 10.1016/j.jhep.2010.07.038 Hillaire, 2002, Idiopathic non-cirrhotic intrahepatic portal hypertension in the West: a re-evaluation in 28 patients, Gut, 51, 275, 10.1136/gut.51.2.275 Nakanuma, 2001, Pathology and pathogenesis of idiopathic portal hypertension with an emphasis on the liver, Pathol Res Pract, 197, 65, 10.1078/0344-0338-5710012 Ziol, 2004, Intrasinusoidal cytotoxic CD8+ T cells in nodular regenerative hyperplasia of the liver, Hum Pathol, 35, 1241, 10.1016/j.humpath.2004.06.016 Chapel, 2008, Common variable immunodeficiency disorders: division into distinct clinical phenotypes, Blood, 112, 277, 10.1182/blood-2007-11-124545 Gathmann, 2014, Clinical picture and treatment of 2212 patients with common variable immunodeficiency, J Allergy Clin Immunol, 134, 116, 10.1016/j.jaci.2013.12.1077 Belkaid, 2014, Role of the microbiota in immunity and inflammation, Cell, 157, 121, 10.1016/j.cell.2014.03.011 Brenchley, 2006, Microbial translocation is a cause of systemic immune activation in chronic HIV infection, Nat Med, 12, 1365, 10.1038/nm1511 Xavier, 2007, Unravelling the pathogenesis of inflammatory bowel disease, Nature, 448, 427, 10.1038/nature06005 Ahmad Kendong, 2021, Gut dysbiosis and intestinal barrier dysfunction: potential explanation for early-onset colorectal cancer, Front Cell Infect Microbiol, 11, 10.3389/fcimb.2021.744606 Yu, 2018, Microbiota dysbiosis and barrier dysfunction in inflammatory bowel disease and colorectal cancers: exploring a common ground hypothesis, J Biomed Sci, 25, 79, 10.1186/s12929-018-0483-8 Berg, 1979, Translocation of certain indigenous bacteria from the gastrointestinal tract to the mesenteric lymph nodes and other organs in a gnotobiotic mouse model, Infect Immun, 23, 403, 10.1128/iai.23.2.403-411.1979 Wiest, 2003, Gastrointestinal disorders of the critically ill. Bacterial translocation in the gut, Best Pract Res Clin Gastroenterol, 17, 397, 10.1016/S1521-6918(03)00024-6 Pinzone, 2012, Microbial translocation in chronic liver diseases, Int J Microbiol, 2012, 10.1155/2012/694629 Tripathi, 2018, The gut-liver axis and the intersection with the microbiome, Nat Rev Gastroenterol Hepatol, 15, 397, 10.1038/s41575-018-0011-z Chopyk, 2020, Contribution of the intestinal microbiome and gut barrier to hepatic disorders, Gastroenterology, 159, 849, 10.1053/j.gastro.2020.04.077 Stehle, 2012, Lipopolysaccharide-binding protein, a surrogate marker of microbial translocation, is associated with physical function in healthy older adults, J Gerontol A Biol Sci Med Sci, 67, 1212, 10.1093/gerona/gls178 Agarwal, 2019, Gastrointestinal manifestations and complications of primary immunodeficiency disorders, Immunol Allergy Clin North Am, 39, 81, 10.1016/j.iac.2018.08.006 Crescenzi, 2019, Liver stiffness assessment by transient elastography suggests high prevalence of liver involvement in common variable immunodeficiency, Dig Liver Dis, 51, 1599, 10.1016/j.dld.2019.05.016 Jorgensen, 2016, Altered gut microbiota profile in common variable immunodeficiency associates with levels of lipopolysaccharide and markers of systemic immune activation, Mucosal Immunol, 9, 1455, 10.1038/mi.2016.18 van Schewick, 2022, Bowel histology of CVID patients reveals distinct patterns of mucosal inflammation, J Clin Immunol, 42, 46, 10.1007/s10875-021-01104-5 Malamut, 2010, The enteropathy associated with common variable immunodeficiency: the delineated frontiers with celiac disease, Am J Gastroenterol, 105, 2262, 10.1038/ajg.2010.214 Daniels, 2007, Gastrointestinal tract pathology in patients with common variable immunodeficiency (CVID): a clinicopathologic study and review, Am J Surg Pathol, 31, 1800, 10.1097/PAS.0b013e3180cab60c Carter, 2013, CVID patients with autoimmunity have elevated T cell expression of granzyme B and HLA-DR and reduced levels of Treg cells, J Clin Pathol, 66, 146, 10.1136/jclinpath-2012-201046 Myneedu, 2021, Autoimmune hepatitis in a patient with common variable immunodeficiency, ACG Case Rep J, 8, 10.14309/crj.0000000000000547 Pollock, 2020, Autoimmune hepatitis in a patient with common variable immunodeficiency, ACG Case Rep J, 7, 10.14309/crj.0000000000000467 Austin, 2004, Nodular regenerative hyperplasia of the liver and coeliac disease: potential role of IgA anticardiolipin antibody, Gut, 53, 1032, 10.1136/gut.2003.036806 European Association for the Study of the, 2015, EASL clinical practice guidelines: autoimmune hepatitis, J Hepatol, 63, 971, 10.1016/j.jhep.2015.06.030 Globig, 2022, Evaluation of laboratory and sonographic parameters for detection of portal hypertension in patients with common variable immunodeficiency, J Clin Immunol, 42, 1626, 10.1007/s10875-022-01319-0 de Franchis, 2022, Faculty. Corrigendum to ‘Baveno VII - renewing consensus in portal hypertension’ [J Hepatol (2022) 959-974], J Hepatol, 77, 271, 10.1016/j.jhep.2022.03.024 Furuichi, 2013, Noninvasive diagnostic method for idiopathic portal hypertension based on measurements of liver and spleen stiffness by ARFI elastography, J Gastroenterol, 48, 1061, 10.1007/s00535-012-0703-z Ahmad, 2019, Spleen stiffness measurements using point shear wave elastography detects noncirrhotic portal hypertension in human immunodeficiency virus, Medicine (Baltimore), 98, 10.1097/MD.0000000000017961 De Gottardi, 2022, Porto-sinusoidal vascular disorder, J Hepatol, 77, 1124, 10.1016/j.jhep.2022.05.033 Glatard, 2012, Obliterative portal venopathy: findings at CT imaging, Radiology, 263, 741, 10.1148/radiol.12111785 Agarwal, 2009, Autoimmunity in common variable immunodeficiency, Curr Allergy Asthma Rep, 9, 347, 10.1007/s11882-009-0051-0 Agarwal, 2013, Diagnosis and treatment of gastrointestinal disorders in patients with primary immunodeficiency, Clin Gastroenterol Hepatol, 11, 1050, 10.1016/j.cgh.2013.02.024 Musumba, 2013, Review article: the association between nodular regenerative hyperplasia, inflammatory bowel disease and thiopurine therapy, Aliment Pharmacol Ther, 38, 1025, 10.1111/apt.12490 Vernier-Massouille, 2007, Nodular regenerative hyperplasia in patients with inflammatory bowel disease treated with azathioprine, Gut, 56, 1404, 10.1136/gut.2006.114363 Seksik, 2011, Incidence of nodular regenerative hyperplasia in inflammatory bowel disease patients treated with azathioprine, Inflamm Bowel Dis, 17, 565, 10.1002/ibd.21330 de Boer, 2005, Nodular regenerative hyperplasia and thiopurines: the case for level-dependent toxicity, Liver Transplant, 11, 1300, 10.1002/lt.20554 Tranah, 2021, Challenges in liver transplantation for common variable immunodeficiency-related liver disease: a case series and systematic review, J Liver Transplant, 4, 100038, 10.1016/j.liver.2021.100038 Tripathi, 2014, Beta-blockers in portal hypertension: new developments and controversies, Liver Int, 34, 655, 10.1111/liv.12360 Li, 2016, Carvedilol for portal hypertension in cirrhosis: systematic review with meta-analysis, BMJ Open, 6, 10.1136/bmjopen-2015-010902 Banares, 1999, Carvedilol, a new nonselective beta-blocker with intrinsic anti- Alpha1-adrenergic activity, has a greater portal hypotensive effect than propranolol in patients with cirrhosis, Hepatology, 30, 79, 10.1002/hep.510300124 Sarin, 2005, Evaluation of endoscopic variceal ligation (EVL) versus propanolol plus isosorbide mononitrate/nadolol (ISMN) in the prevention of variceal rebleeding: comparison of cirrhotic and noncirrhotic patients, Dig Dis Sci, 50, 1538, 10.1007/s10620-005-2877-1 Sarin, 2010, Equal efficacy of endoscopic variceal ligation and propranolol in preventing variceal bleeding in patients with noncirrhotic portal hypertension, Gastroenterology, 139, 1238, 10.1053/j.gastro.2010.06.017 Rodrigues, 2020, Beta-blockers in cirrhosis: evidence-based indications and limitations, JHEP Rep, 2 Boike, 2022, North American practice-based recommendations for transjugular intrahepatic portosystemic shunts in portal hypertension, Clin Gastroenterol Hepatol, 20, 1636, 10.1016/j.cgh.2021.07.018 Vizzutti, 2020, Transjugular intrahepatic portosystemic shunt (TIPS): current indications and strategies to improve the outcomes, Intern Emerg Med, 15, 37, 10.1007/s11739-019-02252-8 Garcia-Pagan, 2020, Where does TIPS fit in the management of patients with cirrhosis?, JHEP Rep, 2 Globig, 2021, International multicenter experience of transjugular intrahepatic portosystemic shunt implantation in patients with common variable immunodeficiency, J Allergy Clin Immunol Pract, 9, 2931, 10.1016/j.jaip.2021.02.056 Zainaldain, 2020, Infectious complications reporting in common variable immunodeficiency: a systematic review and meta-analysis, Oman Med J, 35, 10.5001/omj.2020.64 Bissonnette, 2016, Role of the transjugular intrahepatic portosystemic shunt in the management of severe complications of portal hypertension in idiopathic noncirrhotic portal hypertension, Hepatology, 64, 224, 10.1002/hep.28547 Bettinger, 2016, Procedural and shunt-related complications and mortality of the transjugular intrahepatic portosystemic shunt (TIPSS), Aliment Pharmacol Ther, 44, 1051, 10.1111/apt.13809 Racanelli, 2006, The liver as an immunological organ, Hepatology, 43, S54, 10.1002/hep.21060 Nesseler, 2012, Clinical review: the liver in sepsis, Crit Care, 16, 235, 10.1186/cc11381 Azzu, 2018, Liver transplantation in adults with liver disease due to common variable immunodeficiency leads to early recurrent disease and poor outcome, Liver Transpl, 24, 171, 10.1002/lt.24979 Magaz, 2023, Liver transplantation for porto-sinusoidal vascular liver disorder: long-term outcome, Transplantation, 107, 1330, 10.1097/TP.0000000000004444 Bonatti, 2023, Good long-term outcome following liver transplant in a patient with common variable immunodeficiency syndrome despite multiple infections and recurrent nodular regenerative hyperplasia, Exp Clin Transpl, 21, 66, 10.6002/ect.2022.0067 Apostolov, 2019, Successful liver transplantation in common variable immune deficiency with reversal of hepatopulmonary syndrome, BMJ Case Rep, 12, 10.1136/bcr-2018-226095 Haboubi, 1988, Role of endothelial cell injury in the spectrum of azathioprine-induced liver disease after renal transplant: light microscopy and ultrastructural observations, Am J Gastroenterol, 83, 256 Gane, 1994, Nodular regenerative hyperplasia of the liver graft after liver transplantation, Hepatology, 20, 88, 10.1002/hep.1840200114 Ghabril, 2014, Drug-induced nodular regenerative hyperplasia, Semin Liver Dis, 34, 240, 10.1055/s-0034-1375963 Hadzic, 2000, Correction of the hyper-IgM syndrome after liver and bone marrow transplantation, N Engl J Med, 342, 320, 10.1056/NEJM200002033420504 Gioia, 2020, Causes and management of non-cirrhotic portal hypertension, Curr Gastroenterol Rep, 22, 56, 10.1007/s11894-020-00792-0 Bihl, 2010, Anticoagulant therapy for nodular regenerative hyperplasia in a HIV-infected patient, BMC Gastroenterol, 10, 6, 10.1186/1471-230X-10-6 Abraldes, 2009, Simvastatin lowers portal pressure in patients with cirrhosis and portal hypertension: a randomized controlled trial, Gastroenterology, 136, 1651, 10.1053/j.gastro.2009.01.043 Pollo-Flores, 2015, Three months of simvastatin therapy vs. placebo for severe portal hypertension in cirrhosis: a randomized controlled trial, Dig Liver Dis, 47, 957, 10.1016/j.dld.2015.07.156 Abraldes, 2016, Addition of simvastatin to standard therapy for the prevention of variceal rebleeding does not reduce rebleeding but increases survival in patients with cirrhosis, Gastroenterology, 150, 1160, 10.1053/j.gastro.2016.01.004 Albillos, 2020, The gut-liver axis in liver disease: pathophysiological basis for therapy, J Hepatol, 72, 558, 10.1016/j.jhep.2019.10.003 Ventura, 2014, Bifidobacteria and humans: our special friends, from ecological to genomics perspectives, J Sci Food Agric, 94, 163, 10.1002/jsfa.6356 Poto, 2023, Is there a role for microbiome-based approach in common variable immunodeficiency?, Clin Exp Med, 1 Blaser, 2016, Antibiotic use and its consequences for the normal microbiome, Science, 352, 544, 10.1126/science.aad9358 Bajaj, 2013, Modulation of the metabiome by rifaximin in patients with cirrhosis and minimal hepatic encephalopathy, PLoS One, 8, 10.1371/journal.pone.0060042 Kalambokis, 2012, Rifaximin improves thrombocytopenia in patients with alcoholic cirrhosis in association with reduction of endotoxaemia, Liver Int, 32, 467, 10.1111/j.1478-3231.2011.02650.x Jorgensen, 2019, Rifaximin alters gut microbiota profile, but does not affect systemic inflammation - a randomized controlled trial in common variable immunodeficiency, Sci Rep, 9, 167, 10.1038/s41598-018-35367-7 Belvoncikova, 2022, Gut dysbiosis and fecal microbiota transplantation in autoimmune diseases, Int J Mol Sci, 23 Caira-Chuquineyra, 2022, Fecal microbiota transplantation for people living with human immunodeficiency virus: a scoping review, AIDS Res Hum Retroviruses, 38, 700, 10.1089/aid.2022.0016 Marcella, 2021, Systematic review: the global incidence of faecal microbiota transplantation-related adverse events from 2000 to 2020, Aliment Pharmacol Ther, 53, 33, 10.1111/apt.16148