Liver disease accompanied by enteropathy in common variable immunodeficiency: Common pathophysiological mechanisms

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dc.contributorSistema FMUSP-HC: Faculdade de Medicina da Universidade de São Paulo (FMUSP) e Hospital das Clínicas da FMUSP
dc.contributor.authorLIMA, Fabiana Mascarenhas Souza
dc.contributor.authorTOLEDO-BARROS, Myrthes
dc.contributor.authorALVES, Venancio Avancini Ferreira
dc.contributor.authorDUARTE, Maria Irma Seixas
dc.contributor.authorTAKAKURA, Cleusa
dc.contributor.authorBERNARDES-SILVA, Carlos Felipe
dc.contributor.authorMARINHO, Ana Karolina Barreto Berselli
dc.contributor.authorGRECCO, Octavio
dc.contributor.authorKALIL, Jorge
dc.contributor.authorKOKRON, Cristina Maria
dc.date.accessioned2022-12-21T13:28:01Z
dc.date.available2022-12-21T13:28:01Z
dc.date.issued2022
dc.description.abstractCommon variable immunodeficiency (CVID) is one of the inborn errors of immunity that have the greatest clinical impact. Rates of morbidity and mortality are higher in patients with CVID who develop liver disease than in those who do not. The main liver disorder in CVID is nodular regenerative hyperplasia (NRH), the cause of which remains unclear and for which there is as yet no treatment. The etiology of liver disease in CVID is determined by analyzing the liver injury and the associated conditions. The objective of this study was to compare CVID patients with and without liver-spleen axis abnormalities in terms of clinical characteristics, as well as to analyze liver and duodenal biopsies from those with portal hypertension (PH), to elucidate the pathophysiology of liver injury. Patients were divided into three groups: Those with liver disease/PH, those with isolated splenomegaly, and those without liver-spleen axis abnormalities. Clinical and biochemical data were collected. Among 141 CVID patients, 46 (32.6%) had liver disease/PH; 27 (19.1%) had isolated splenomegaly; and 68 (48.2%) had no liver-spleen axis abnormalities. Among the liver disease/PH group, patients, even those with mild or no biochemical changes, had clinical manifestations of PH, mainly splenomegaly, thrombocytopenia, and esophageal varices. Duodenal celiac pattern was found to correlate with PH (p < 0.001). We identified NRH in the livers of all patients with PH (n = 11). Lymphocytic infiltration into the duodenal mucosa also correlated with PH. Electron microscopy of liver biopsy specimens showed varying degrees of lymphocytic infiltration and hepatocyte degeneration, which is a probable mechanism of lymphocyte-mediated cytotoxicity against hepatocytes and enterocytes. In comparison with the CVID patients without PH, those with PH were more likely to have lymphadenopathy (p < 0.001), elevated beta(2)-microglobulin (p < 0.001), low B-lymphocyte counts (p < 0.05), and low natural killer-lymphocyte counts (p < 0.05). In CVID patients, liver disease/PH is common and regular imaging follow-up is necessary. These patients have a distinct immunological phenotype that may predispose to liver and duodenal injury from lymphocyte-mediated cytotoxicity. Further studies could elucidate the cause of this immune-mediated mechanism and its treatment options.eng
dc.description.indexMEDLINEeng
dc.description.sponsorshipBrazilian Conselho Nacional de Desenvolvimento Cientifico e Tecnologico (CNPq, National Council for Scientific and Technological Development)
dc.description.sponsorshipInstituto Nacional de Ciencia e Tecnologia de Investigacao em Imunologia (National Institute of Science and Technology for Investigation in Immunology-III/INCT)
dc.description.sponsorshipSao Paulo, SP, Brazil
dc.description.sponsorshipConselho Nacional de Desenvolvimento Cientifico e Tecnologico (National Council for Scientific and Technological Development) - CNPq - Brazil
dc.description.sponsorshipINCT/CNPq-iii [465.434/2014-2]
dc.identifier.citationFRONTIERS IN IMMUNOLOGY, v.13, article ID 933463, 19p, 2022
dc.identifier.doi10.3389/fimmu.2022.933463
dc.identifier.issn1664-3224
dc.identifier.urihttps://observatorio.fm.usp.br/handle/OPI/50686
dc.language.isoeng
dc.publisherFRONTIERS MEDIA SAeng
dc.relation.ispartofFrontiers in Immunology
dc.rightsopenAccesseng
dc.rights.holderCopyright FRONTIERS MEDIA SAeng
dc.subjectinborn errors of immunity (IEI)eng
dc.subjectprimary immunodeficiency (PID)eng
dc.subjectcommon variable immunodeficiency (CVID)eng
dc.subjectportal hypertensioneng
dc.subjectliver diseaseeng
dc.subjectnodular regenerative hyperplasia (NRH)eng
dc.subjectenteropathyeng
dc.subjectduodenal celiac patterneng
dc.subject.othernodular regenerative hyperplasiaeng
dc.subject.otherchronic norovirus infectioneng
dc.subject.otherprimary biliary-cirrhosiseng
dc.subject.otherceliac-diseaseeng
dc.subject.otherportal-hypertensioneng
dc.subject.otherautoimmune hepatitiseng
dc.subject.otherimmune-deficiencyeng
dc.subject.otherdisorderseng
dc.subject.otherbeta-2-microglobulineng
dc.subject.othermanifestationseng
dc.subject.wosImmunologyeng
dc.titleLiver disease accompanied by enteropathy in common variable immunodeficiency: Common pathophysiological mechanismseng
dc.typearticleeng
dc.type.categoryoriginal articleeng
dc.type.versionpublishedVersioneng
dspace.entity.typePublication
hcfmusp.citation.scopus7
hcfmusp.contributor.author-fmusphcFABIANA MASCARENHAS SOUZA LIMA
hcfmusp.contributor.author-fmusphcMYRTHES ANNA MARAGNA TOLEDO BARROS
hcfmusp.contributor.author-fmusphcVENANCIO AVANCINI FERREIRA ALVES
hcfmusp.contributor.author-fmusphcMARIA IRMA SEIXAS DUARTE
hcfmusp.contributor.author-fmusphcCLEUSA FUMICA HIRATA TAKAKURA
hcfmusp.contributor.author-fmusphcCARLOS FELIPE BERNARDES SILVA
hcfmusp.contributor.author-fmusphcANA KAROLINA BARRETO BERSELLI MARINHO
hcfmusp.contributor.author-fmusphcOCTAVIO GRECCO
hcfmusp.contributor.author-fmusphcJORGE ELIAS KALIL FILHO
hcfmusp.contributor.author-fmusphcCRISTINA MARIA KOKRON
hcfmusp.description.articlenumber933463
hcfmusp.description.volume13
hcfmusp.origemWOS
hcfmusp.origem.pubmed36341360
hcfmusp.origem.scopus2-s2.0-85141186866
hcfmusp.origem.wosWOS:000879893200001
hcfmusp.publisher.cityLAUSANNEeng
hcfmusp.publisher.countrySWITZERLANDeng
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