PAZIENTE IMMUNOTOLLERANTE E PORTATORE INATTIVO Dr. Giuliano Alagna StorianaturaleHBV Replicazione viarle Immunità/risposta dell’ospite Liver Failure 3% INFEZIONE ACUTA INFEZIONE CRONICA 5% INACTIVE CARRIER Cirrosi 30% Liver Cancer 3% MORTE ALT HBVDNA HBeAg Anatomiapatologica Immuno-tolerant Normali Elevate:>1milioneIU/ml POS Minimainfiammazioneofibrosi HBeAg+ Immuneac:ve Elevete Elevate>20,000IU/ml POS Infiammazionemoderatasevera ofibrosi Inac:veCHB Normali Bassoononrilevabile <2,000IU/ml NEG Minimanecroinfiammazionema fibrosivariabile HBeAg-immune reac:va:on Elevate Elevate >2,000IU/ml NEG Infiammazionedamoderataa severaofibrosi I fase: “immuno tolerant”: HBeAg+, alti livelli di replicazione virale (HBV DNA +++), livelli normali o bassi di transaminasi lenta progressione della di fibrosi con scarsi segni di infiammazione/necrosi. Questa fase è più frequente e lunga nei pazienti con infezione perinatale o contratta nei primi anni di vita. Basso tasso di perdita dell’ Hbe. Ifase:“immunotolerant”:HBeAg+,alXlivellidireplicazionevirale(HBV Fase altamente contagiosa DNA+++),livellinormaliobassiditransaminasilentaprogressionedella difibrosiconscarsisegnidiinfiammazione/necrosi.Questafaseèpiù IIfrequenteelunganeipazienXconinfezioneperinataleocontrabanei fase: “immuno reactive phase”: HbeAg+, bassa replica virale con basso HBV DNA, livelli aumentati o fluttuanti di transaminasi, segni di moderata o severa necrosi ed primiannidivita.Bassotassodiperditadell’HBe.Fasealtamente infiammazione epatica e più rapida progressione verso la fibrosi rispetto alla fase I. Piutipica dell’ età adulta contagiosa III fase: “inactive HBV carrier state”: può seguire la sieroconversione da HBeAg+ ad HbeAg negativo. E' caratterizzata da bassissimi valori di HBV DNA e livelli normali di aminotransferasi. Questo stadio è associato ad una bassa probabilità di progressione verso la cirrosi e l'HCC…. IIIfase:“inacXveHBVcarrierstate”:puòseguirelasieroconversioneda HBeAg+adanXHBeAg.E'caraberizzatadabassissimivaloridiHBVDNA IV fase: “HBeAg-negative CHB” .: E' caratterizzata da periodiche riattivazioni con elivellinormalidiaminotransferasi.E’necessariounfollowupminimo fluttuazioni sia dell'HBV DNA che delle transaminasi. diunannomonitorizzandoleALT(<40UI/ML)el’HBVDNA(<2000U/ Vml).Questostadioèassociatoadunabassaprobabilitàdiprogressione fase: “HBsAg negative phase” dopo la perdita dell'antigene di superficie bassi valori di HBV DNA possono persistere. La perdita dell' HBsAg è associata ad un miglioramento versolacirrosiel'HCC dell' outcome con una riduzione del rischio di progressione verso la cirrosi e verso l'HCC. IN QUESTI PAZIENTI L'IMMUNOSOPPRESSIONE PUO' PORTARE ALLA RIATTIVAZIONE DELL'HBV HBV carriers (HBsAg+): attivo HBeAg o anti HBe+ HBV DNA>20000 UI/ml inattivo HBeAg – Anti HBe+HBV DNA<2000 UI/ml Occult HBV carriers (HBsAg-): HBV DNA rilevabile come CCC DNA o HBV DNA nel siero molto basso IMMUNOTOLLERANTE need for treatment. Of note, some persons will be in the “gray zones,” meaning that their HBV DNA and ALT evels do not fall into the same phase. Longitudinal ollow-up of ALT and HBV DNA levels and/or assessment of liver histology can serve to clarify the phase of nfection. i. Immune-tolerant phase: In this highly replicative/ low inflammatory phase, HBV DNA levels are elevated, ALT levels are normal (<19 U/L for females and <30 U/L for males), and biopsies are without signs of significant inflammation or fibrosis. The duration of this phase is highly variable, but longest in those who are infected perinatally. With increasing age, there is an increased likelihood of transitioning from immune-tolerant to the HBeAg-positive immuneactive phase. ii. HBeAg-positive immune-active phase: Elevated ALT and HBV DNA levels in conjunction with liver injury characterize this phase. Median age of onset is 30 years among those infected at a young age. The hallmark of transition from the HBeAgpositive immune-active to -inactive phases is HBeAg seroconversion. The rate of spontaneous Ø HBVDNAelevato Ø ALTnormali(F<19UI/ml; M<30UI/ml) Ø Nofibrosi Ø Fasepiùfrequentenelleprime duedecadi Guarigione Formacronica 90-95% 5-10% Bambinie AdolescenX 40% 60% NeonaX 10% 90% AdulX PerinatalvspostnatalacquisiXon(1) Journal of Hepatology 48 (2008) 335–352 www.elsevier.com/locate/jhep Review Natural history of chronic hepatitis B: Special emphasis on disease progression and prognostic factorsq 1,* 2 Giovanna Fattovich , Flavia Bortolotti , Francesco Donato 1 3 Department of Surgical and Gastroenterological Sciences, University of Verona, Piazzale L.A. Scuro, 10, Verona 37134, Italy 2 Fifth Medical Clinic, University of Padova, Padova, Italy 3 Institute of Hygiene, Epidemiology and Public Health, University of Brescia, Brescia, Italy The natural history of chronic hepatitis B virus (HBV) infection and disease is complex and highly variable. We review the natural history of chronic hepatitis B with emphasis on the rates of disease progression and factors influencing the course of the liver disease. Chronic hepatitis B is characterized by an early replicative phase (HBeAg positive chronic hepatitis) and a late low or non-replication phase with HBeAg seroconversion and liver disease remission (inactive carrier state). Most patients become inactive carriers after spontaneous HBeAg seroconversion with good prognosis, but progression to HBeAg negative chronic hepatitis due to HBV variants not expressing HBeAg occurs at a rate of 1–3 per 100 person years following HBeAg seroconversion. The incidence of cirrhosis appears to be about 2-fold higher in HBeAg negative compared to HBeAg positive chronic hepatitis. In the cirrhotic patient the 5-year cumulative risk of developing hepatocellular carcinoma is 17% in East Asia and 10% in the Western Europe and the United States and the 5-year liver related death rate is 15% in Europe and 14% in East Asia. There is a growing understanding of viral, host and environmental factors influencing disease progression, which ultimately could improve the management of chronic hepatitis B. ! 2007 European Association for the Study of the Liver. Published by Elsevier B.V. All rights reserved. Keywords: Chronic hepatitis B; Natural history; Prognostic factors 1. Introduction Chronic infection with hepatitis B virus (HBV) currently affects about 400 million people, particularly in developing countries, and it is estimated that worldwide over 200,000 and 300,000 chronic HBV carriers die each year from cirrhosis and hepatocellular carcinoma (HCC), respectively [1,2]. The natural history of chronic HBV infection and disease is variable and complex and has still not been completely defined. A careful understanding of the clinical outcomes and factors affecting Associate Editor: R.P. Perrillo q The authors declare that they do not have anything to disclose regarding funding from industries or conflict of interest with respect to this manuscript * Corresponding author. Tel./fax: +39 045 8124205. E-mail address: [email protected] (G. Fattovich). Perinatale Ø InasiaibambiniinfebaXinepocaperinatale,presentanonormalivaloridiALT conminimodannoepaXco(immunotolerantphase),essendoperaltro asintomaXci disease progression is important in the management of chronic hepatitis B. This article reviews the natural history of chronic hepatitis B, with emphasis on summarizing the available data to estimate the rates of disease progression according to the stage of the disease and factors influencing its course. 2. Phases of chronic HBV infection The likelihood of developing chronic HBV infection is higher in individuals infected perinatally (90%) or during childhood (20–30%), when the immune system is thought to be immature, compared with immunocompetent subjects infected during adulthood (<1%). The phases of chronic HBV infection described herein refer to patients infected early in life. The natural course of chronic HBV infection can be divided into four phases based on the 0168-8278/$32.00 ! 2007 European Association for the Study of the Liver. Published by Elsevier B.V. All rights reserved. doi:10.1016/j.jhep.2007.11.011 Ø LapercentualedisieroconversioneadanXHBeèmoltovariabileaseconda deglistudi(1,2)essendocompresatrail10edil75%a10/13annid’età Ø QuandosihalasieroconversionedaHBeadanXHBe,siassisteall’incremento delleALTedallariduzionedell’HBVDNA(puntonodale) Ø Studilongitudinalidocumentanochelasieroconversioneconduceallostadio diportatoreinamvo(clearancedell’HBsAg0.6%/anno);neibambiniincuisiha unaseveranecroinfiammazionedurantelasieroconversioneanXHBesipuò avereunarapidaprogressioneversolacirrosielosviluppodiHCC 1ChuCM,LiawYF.ChronichepaXXsBvirusinfecXonacquiredinchildhood:specialemphasisonprognosXcandtherapeuXcimplicaXonof delayedHBeAgseroconversion.JViralHepat2007;14:147–152. 2MarxG,MarXnSR,ChicoineJF,AlvarezF.Long-termfollow-upofchronichepaXXsBvirusinfecXoninchildrenofdifferentethnicorigin.J InfectDis2002;186:295–301. Journal of Hepatology 48 (2008) 335–352 www.elsevier.com/locate/jhep Review Natural history of chronic hepatitis B: Special emphasis on disease progression and prognostic factorsq PerinatalvspostnatalacquisiXon(2) Giovanna Fattovich1,*, Flavia Bortolotti2, Francesco Donato3 1 Department of Surgical and Gastroenterological Sciences, University of Verona, Piazzale L.A. Scuro, 10, Verona 37134, Italy 2 Fifth Medical Clinic, University of Padova, Padova, Italy 3 Institute of Hygiene, Epidemiology and Public Health, University of Brescia, Brescia, Italy The natural history of chronic hepatitis B virus (HBV) infection and disease is complex and highly variable. We review the natural history of chronic hepatitis B with emphasis on the rates of disease progression and factors influencing the course of the liver disease. Chronic hepatitis B is characterized by an early replicative phase (HBeAg positive chronic hepatitis) and a late low or non-replication phase with HBeAg seroconversion and liver disease remission (inactive carrier state). Most patients become inactive carriers after spontaneous HBeAg seroconversion with good prognosis, but progression to HBeAg negative chronic hepatitis due to HBV variants not expressing HBeAg occurs at a rate of 1–3 per 100 person years following HBeAg seroconversion. The incidence of cirrhosis appears to be about 2-fold higher in HBeAg negative compared to HBeAg positive chronic hepatitis. In the cirrhotic patient the 5-year cumulative risk of developing hepatocellular carcinoma is 17% in East Asia and 10% in the Western Europe and the United States and the 5-year liver related death rate is 15% in Europe and 14% in East Asia. There is a growing understanding of viral, host and environmental factors influencing disease progression, which ultimately could improve the management of chronic hepatitis B. ! 2007 European Association for the Study of the Liver. Published by Elsevier B.V. All rights reserved. Keywords: Chronic hepatitis B; Natural history; Prognostic factors 1. Introduction Chronic infection with hepatitis B virus (HBV) currently affects about 400 million people, particularly in developing countries, and it is estimated that worldwide over 200,000 and 300,000 chronic HBV carriers die each year from cirrhosis and hepatocellular carcinoma (HCC), respectively [1,2]. The natural history of chronic HBV infection and disease is variable and complex and has still not been completely defined. A careful understanding of the clinical outcomes and factors affecting Associate Editor: R.P. Perrillo q The authors declare that they do not have anything to disclose regarding funding from industries or conflict of interest with respect to this manuscript * Corresponding author. Tel./fax: +39 045 8124205. E-mail address: [email protected] (G. Fattovich). Postnatal disease progression is important in the management of chronic hepatitis B. This article reviews the natural history of chronic hepatitis B, with emphasis on summarizing the available data to estimate the rates of disease progression according to the stage of the disease and factors influencing its course. Ø Lamaggiorpartedeibambiniconinfezioneacquisitaarrivaall’osservazionein unafasediincrementodelleALTconmalamaamva 2. Phases of chronic HBV infection The likelihood of developing chronic HBV infection is higher in individuals infected perinatally (90%) or during childhood (20–30%), when the immune system is thought to be immature, compared with immunocompetent subjects infected during adulthood (<1%). The phases of chronic HBV infection described herein refer to patients infected early in life. The natural course of chronic HBV infection can be divided into four phases based on the 0168-8278/$32.00 ! 2007 European Association for the Study of the Liver. Published by Elsevier B.V. All rights reserved. doi:10.1016/j.jhep.2007.11.011 Ø SieroconversioneadanXHBetrail14-16%/annoduranteiprimi10anni Ø InunostudiolongitudinaleItaliano(*)il95%dibambiniandaXincontroa sieroconversioneHBesonodiventaXportatoriinamviprimadelraggiungimento dell’etàadulta *BortolomF,GuidoM,BartolacciS,CadrobbiP,CrivellaroC,NoventaF,etal.ChronichepaXXsBinchildrenavereanXgen seroclearance:finalreportofa29yearlongitudinalstudy.Hepatology2006;43:556–562. Table 2. Among 17 studies of inter 47 Ageand overRationale 40 years is associa years. Evidence tolerant adults, only two examined Recommendations Quality/Certainly of Evidence: Moderate The evidence profile is summ lihood of significant histological Technical Remark than ULNs, whereas most used AL Strength of Recommendation: Strong Table 2. Among 17 studies of inte positivefor persons with All normal ALT The AASLD status recommends against inclusion. were RCTsle 1.2A.Immune-tolerant should be definedantiviral by ALT ULNs tolerant adults, only two examined Technical Remark thanofULNs, whereas 24-48 weeks formost IFN orused 48 wA therapy adults with CHB.!19 U/L tion levelsforutilizing !30immune-tolerant U/L for men and ULNs forand inclusion. All werefollow RCT 1. Immune-tolerant status should defined by ALT Evidence 12 months of Rationale post-treatment for women as ULNs rather thanbe local laboratory of 24-48 weeks for IFN or 48 Quality/Certainly Evidence: Moderate levels utilizing of !30 U/L for men and !19 U/L tion The evidence profile is summa HBeAg loss and seroconversion as t ULNs. 12 months of post-treatment follo for women as ULNs rather than local laboratory Strength of Recommendation: Strong Table 2. Among interv whereas onlyand two17seroconversion studies ofevaluate HBeAg loss as ULNs. 2B. The AASLD suggests that ALT levels be tested tolerant whereascomparing only only twoantiviral studies evaluat studies therapy adults, two examined 2B. The AASLD suggests that ALT levels be tested at least Remark every 6 months for adults with immune- ment studies comparing antiviral therap Technical were thewhereas primarymost studies ULNs, usedinform AL at least every 6 months for adults with immune- than ment were the primary studies info tolerant CHB to monitor for potential transition to CHB to monitor for potential transition to ULNs dation. The remaining 1212RCTs studies for inclusion. All were dation. The remaining studi 1.tolerant Immune-tolerant status should be defined by ALT immune-active or -inactive CHB. immune-active or -inactive CHB. comparisons antiviral comparisons ofdifferent different antiviral tion of 24-48 ofweeks for IFN or 48the wth levels utilizing !30 U/L for men and !19 U/L Comparedtotountreated/placebo untreated/placebo Quality/Certainly Very low low Compared Quality/Certainly of of Evidence: Evidence: Very 12 months of post-treatment follow for women as ULNs rather than local laboratory ralral therapy therapy resulted in a signifi Strength of Recommendation: Conditional resulted in a95% significa Strength of Recommendation: Conditional HBeAg loss (RR, 2.69; HBeAg loss and seroconversion asCI:th ULNs. 2C. The AASLD suggests antiviral therapy in the HBeAg loss (RR, (RR, 2.22; 2.69;95% 95%CI: CI:11 conversion onlyby(RR, two studies evaluate 2C.group The AASLD antiviral in the whereas select of adultssuggests >40 years of agetherapy with normal of results treatment type conversion 2.22; 95% CI:(IFN 1.2 2B. The AASLD suggests that ALT levels be tested ALT and elevated HBV DNA ("1,000,000 IU/mL) select group of adults >40 years of age with normal studies comparing therapy dine) yielded RR antiviral that type included of results by treatment (IFN1a and liver biopsy showing significant necroinflammaatALT leastandevery 6 months for adults with immuneferentwere from controls). T elevated HBV DNA ("1,000,000 IU/mL) ment theuntreated primary studies inform tion or fibrosis. dine) yielded RR that included 1 ( low-to-moderate quality and the tolerant CHB to monitor for potential transition to and liver biopsy showing significant necroinflamma- dation. Thebaseline remaining 12 studies ferent from untreated Th sons with ALTcontrols). values less Quality/Certainly of Evidence: Very low immune-active or -inactive CHB. tion or fibrosis. low to low quality. comparisons of different Strength of Recommendation: Conditional low-to-moderate qualityantiviral and thetheR There are no studies demons Compared to untreated/placebo sons with baseline ALT values less t Quality/Certainly of Evidence: Very low Quality/Certainly of Evidence: Very low therapy is beneficial in reducing ra Technical Remark toliver-related low quality. rallow resulted in ina persons significa StrengthofofRecommendation: Recommendation:Conditional Conditional Strength andtherapy death 1. Moderate-to-severe necroinflammation or fibrosis CHB. Finite duration Thereloss are(RR, notreatment studies95% demonst HBeAg 2.69; CI: 1 therapy for adults with immune-tolerant CHB. INACTIVECARRIER q Qualisonoicriterioggemviperindividuarlo? q Qualeèilrischiodiprogressionedimalamae sviluppoHCC? q Rischiodiriamvazione q QualeXpodifollowupbisognaapplicareinquesX pazienX? Ø L’interazionetravirusedimmunitàdell’ospitespiegaladinamicitàdelle fasidimalama Ø Lacompressionedellasuddebadinamicitàdellastorianaturalehaindobo adunavariazionedellaterminologiauXlizzata 1960 Healtycarrier AsyntomaXcHBsAgcarrier InacXveHBsAgcarrier InacXvecarrierstate 2016 LowreplicaXvechronicHBVInfecXon(APASL) Qualisonoicriterioggemviperindividuarlo? ü ALT ü LivelliHBVDNA ü IstologiaepaXca? ü ALT Clinical Practice Guidelines EASL Clinical Practice Guidelines: Management of chronic hepatitis B virus infection European Association for the Study of the Liver⇑ Introduction Our understanding of the natural history of hepatitis B virus (HBV) infection and the potential for therapy of the resultant disease is continuously improving. New data have become available since the previous EASL Clinical Practice Guidelines (CPGs) prepared in 2008 and published in early 2009 [1]. The objective of this manuscript is to update the recommendations for the optimal management of chronic HBV infection. The CPGs do not fully address prevention including vaccination. In addition, despite the increasing knowledge, areas of uncertainty still exist and therefore clinicians, patients, and public health authorities must continue to make choices on the basis of the evolving evidence. been increasing over the last decade as a result of aging of the HBV-infected population and predominance of specific HBV genotypes and represents the majority of cases in many areas, including Europe [4,9,10]. Morbidity and mortality in CHB are linked to persistence of viral replication and evolution to cirrhosis and/or hepatocellular carcinoma (HCC). Longitudinal studies of untreated patients with CHB indicate that, after diagnosis, the 5-year cumulative incidence of developing cirrhosis ranges from 8% to 20%. The 5-year cumulative incidence of hepatic decompensation is approximately 20% for untreated patients with compensated cirrhosis [2–4,11–13]. Untreated patients with decompensated cirrhosis have a poor prognosis with a 14–35% probability of survival at 5 years [2–4,12]. The worldwide incidence of HCC has increased, mostly due to persistent HBV and/ or HCV infections; presently it constitutes the fifth most common cancer, representing around 5% of all cancers. The annual incidence of HBV-related HCC in patients with CHB is high, ranging from 2% to 5% when cirrhosis is established [13]. However, the incidence of HBV related HCC appears to vary geographically and correlates with the underlying stage of liver disease and possibly exposure to environmental carcinogens such as aflatoxin. Population movements and migration are currently changing the prevalence and incidence of the disease in several low endemic countries in Europe and elsewhere. Substantial healthcare resources will be required for control of the worldwide burden of disease. Aminimumfollow-upof1yearwithalanineaminotransferase(ALT)levelsatleast every3–4monthsandserumHBVDNAlevelsisrequiredbeforeclassifyingapaXent asinacXveHBVcarrier.ALTlevelsshouldremainpersistentlywithinthenor-mal Context rangeaccordingtotradiXonalcut-offvalues(approximately40IU/ml) Epidemiology and public health burden Approximately one third of the world’s population has serological evidence of past or present infection with HBV and 350–400 million people are chronic HBV surface antigen (HBsAg) carriers. The spectrum of disease and natural history of chronic HBV infection are diverse and variable, ranging from an inactive carrier state to progressive chronic hepatitis B (CHB), which may evolve to cirrhosis and hepatocellular carcinoma (HCC) [2–4]. HBV-related end stage liver disease or HCC are responsible for over 0.5–1 million deaths per year and currently represent 5–10% of cases of Natural history ü ALT Digestive and Liver Disease 43S (2011) S8–S14 Contents lists available at ScienceDirect Digestive and Liver Disease journal homepage: www.elsevier.com/locate/dld Ø on MolXdeglistudicondomperindividuare Natural history of chronic HBV infection: special emphasis the prognostic implications of the inactive carrier state versus chroniceventualirischilegaXallostatodiportatore hepatitis inamvosonostaXviziaXdalfabocheèstata a, 1 b,1 a b Erica Villa *, Giovanna Fattovich , Antonella Mauro , Michela Pasino considerataun’unicamisurazionedelleALT a Gastroenterology and Liver Clinic, Azienda Ospedaliero-Universitaria, University of Modena and Reggio Emilia, Modena, Italy b Gastroenterology Clinic, Department of Medicine, Azienda Ospedaliero-Universitaria, University of Verona, Verona, Italy Abstract Ø Glistudicondomconabentomonitoraggiodelle ALThannoevidenziatocomepiùdell’85%dei pazienXritenuXcarrier,loeranoeffemvamente inaccordoconl’esameistologico The evaluation of the natural history of chronic hepatitis B virus (HBV) infection requires the precise definition of the various clinical conditions that can be encountered (i.e. inactive carrier state or subject with liver disease activity). This can be achieved by repeat monitoring of ALT, serum HBV-DNA levels (over a period of at least 1 year, according to international guidelines) and/or evaluation of HBsAg titre. Liver biopsy may offer additional information although it is not mandatory. Overall, the natural history of the true inactive carrier is benign: reactivation of hepatitis, especially in Western countries, is rare and is usually due to co-factors (like alcohol or drugs); spontaneous HBsAg loss is frequent (around 1% per year) and HCC development rare. On the other hand, in patients with chronic hepatitis B or cirrhosis, the risk of reactivation, of HCC development and of liver-related mortality is much higher, especially in Eastern countries, and should therefore lead to antiviral therapy. © 2010 Editrice Gastroenterologica Italiana S.r.l. Published by Elsevier Ltd. All rights reserved. Keywords: Chronic hepatitis; Hepatitis B virus; Inactive carrier state; Prognosis During the last few years there has been a substantial mod- carrier”. Although several studies on the natural history of implications. Indeed, several conditions have to be fulfilled for of an “inactive carrier state” rather than an “inactive carrier”, NaturalhistoryofchronicHBVcarriersinNorthernItaly:morbidityandmortalityaver30years.MannoM,Camma`C,SchepisF,etal. ification of the nomenclature regarding Hepatitis B surface patients with chronic HBV infection have been published, Gastroenterology2004;127:756−63. Antigen (HBsAg) carriers with no or scarce evidence of active a comprehensive analysis is hampered by the different DeFranchisR,MeucciG,VecchiM,etal.ThenaturalhistoryofasymptomaXchepaXXsBsurfaceanXgencarriers.AnnInternMed1993 liver disease. The definition of “healthy carrier” used in the study designs (case–control, cross-sectional, longitudinal) and 1960s has been changed to “asymptomatic HBsAg carrier”, the heterogeneity of the patient populations relative to the VilleneuveJP,DesrochersM,Infante-RivardC,etal.Along-termfollow-upstudyofasymptomaXchepaXXsBsurfaceanXgen-posiXvecarriersin and recently this has been further modified to “inactive clinical setting (inactive carrier, asymptomatic carrier, chronic Montreal.Gastroenterology1994 HBsAg carrier”. This progressive modification is more than hepatitis). Available data have called into question the concept a mere semantic process, as it has substantial clinical of inactive carrier, and proposals have been made to speak Long-termoutcomeofhepaXXsBeanXgen-negaXvehepaXXsB surfaceanXgencarriersinrelaXontochangesofalanine aminotransferaselevelsoverXme 4376HBsAg+,HBeAg-“carrier” ALTvalutateogni3mesi/annoper3anni 1720pazienX(46.8%)neisuccessivitrediciannihannoavutounincrementodelle transaminasi Transaminasipersistentementenormalicorrelanoconun’eccellenteprognosi Unincremento2ULNduranteilFUèassociatoadun’aumentatamorbiditàemortalità Unincremento2ULNpuòessereconsideratoilcut-offdecisionaleriguardolaterapia TaiDietal.Hepatology.2009Jun;49(6):1859-67 ü ALT Clinical Practice Guidelines EASL Clinical Practice Guidelines: Management of chronic hepatitis B virus infection European Association for the Study of the Liver⇑ Introduction Our understanding of the natural history of hepatitis B virus (HBV) infection and the potential for therapy of the resultant disease is continuously improving. New data have become available since the previous EASL Clinical Practice Guidelines (CPGs) prepared in 2008 and published in early 2009 [1]. The objective of this manuscript is to update the recommendations for the optimal management of chronic HBV infection. The CPGs do not fully address prevention including vaccination. In addition, despite the increasing knowledge, areas of uncertainty still exist and therefore clinicians, patients, and public health authorities must continue to make choices on the basis of the evolving evidence. been increasing over the last decade as a result of aging of the HBV-infected population and predominance of specific HBV genotypes and represents the majority of cases in many areas, including Europe [4,9,10]. Morbidity and mortality in CHB are linked to persistence of viral replication and evolution to cirrhosis and/or hepatocellular carcinoma (HCC). Longitudinal studies of untreated patients with CHB indicate that, after diagnosis, the 5-year cumulative incidence of developing cirrhosis ranges from 8% to 20%. The 5-year cumulative incidence of hepatic decompensation is approximately 20% for untreated patients with compensated cirrhosis [2–4,11–13]. Untreated patients with decompensated cirrhosis have a poor prognosis with a 14–35% probability of survival at 5 years [2–4,12]. The worldwide incidence of HCC has increased, mostly due to persistent HBV and/ or HCV infections; presently it constitutes the fifth most common cancer, representing around 5% of all cancers. The annual incidence of HBV-related HCC in patients with CHB is high, ranging from 2% to 5% when cirrhosis is established [13]. However, the incidence of HBV related HCC appears to vary geographically and correlates with the underlying stage of liver disease and possibly exposure to environmental carcinogens such as aflatoxin. Population movements and migration are currently changing the prevalence and incidence of the disease in several low endemic countries in Europe and elsewhere. Substantial healthcare resources will be required for control of the worldwide burden of disease. Aminimumfollow-upof1yearwithalanineaminotransferase(ALT)levels atleastevery3–4monthsandserumHBVDNAlevelsisrequiredbefore classifyingapa:entasinac:veHBVcarrier. Context Epidemiology and public health burden Approximately one third of the world’s population has serological evidence of past or present infection with HBV and 350–400 million people are chronic HBV surface antigen (HBsAg) carriers. The spectrum of disease and natural history of chronic HBV infection are diverse and variable, ranging from an inactive carrier state to progressive chronic hepatitis B (CHB), which may evolve to cirrhosis and hepatocellular carcinoma (HCC) [2–4]. HBV-related end stage liver disease or HCC are responsible for over 0.5–1 million deaths per year and currently represent 5–10% of cases of liver transplantation [5–8]. Host and viral factors, as well as coinfection with other viruses, in particular hepatitis C virus (HCV), hepatitis D virus (HDV), or human immunodeficiency virus BruneboMR,Hepatology1989;CPG,EASL2012 (HIV) together with other co-morbidities including alcohol abuse Liuj1Hepatology.2016 and obesity, can affect the natural course of HBV infection as well Ø HBVDNA<2000UI/mL Ø HBsAg<1000UI/mL Natural history ü HBVDNA Chronic HBV infection is a dynamic process. The natural history of chronic HBV infection can be schematically divided into five phases, which are not necessarily sequential. Digestive and Liver Disease 43S (2011) S8–S14 Contents lists available at ScienceDirect Digestive and Liver Disease journal homepage: www.elsevier.com/locate/dld ü ISTOLOGIA Natural history of chronic HBV infection: special emphasis on the prognostic implications of the inactive carrier state versus chronic hepatitis Erica Villaa, 1*, Giovanna Fattovichb,1, Antonella Mauroa , Michela Pasinob a Gastroenterology and Liver Clinic, Azienda Ospedaliero-Universitaria, University of Modena and Reggio Emilia, Modena, Italy b Gastroenterology Clinic, Department of Medicine, Azienda Ospedaliero-Universitaria, University of Verona, Verona, Italy Abstract The evaluation of the natural history of chronic hepatitis B virus (HBV) infection requires the precise definition of the various clinical conditions that can be encountered (i.e. inactive carrier state or subject with liver disease activity). This can be achieved by repeat monitoring of ALT, serum HBV-DNA levels (over a period of at least 1 year, according to international guidelines) and/or evaluation of HBsAg titre. Liver biopsy may offer additional information although it is not mandatory. Overall, the natural history of the true inactive carrier is benign: reactivation of hepatitis, especially in Western countries, is rare and is usually due to co-factors (like alcohol or drugs); spontaneous HBsAg loss is frequent (around 1% per year) and HCC development rare. On the other hand, in patients with chronic hepatitis B or cirrhosis, the risk of reactivation, of HCC development and of liver-related mortality is much higher, especially in Eastern countries, and should therefore lead to antiviral therapy. © 2010 Editrice Gastroenterologica Italiana S.r.l. Published by Elsevier Ltd. All rights reserved. Ø Pochistudi*hannoriportatodaXriguardol’istologianeiportatoriinamvi; difabosoloil3%presenterebbeun’epaXtecronicamoderata Keywords: Chronic hepatitis; Hepatitis B virus; Inactive carrier state; Prognosis During the last few years there has been a substantial modification of the nomenclature regarding Hepatitis B surface Antigen (HBsAg) carriers with no or scarce evidence of active liver disease. The definition of “healthy carrier” used in the 1960s has been changed to “asymptomatic HBsAg carrier”, and recently this has been further modified to “inactive HBsAg carrier”. This progressive modification is more than a mere semantic process, as it has substantial clinical implications. Indeed, several conditions have to be fulfilled for a subject to be included in this subgroup of inactive hepatitis B virus (HBV) carriers, namely: hepatitis B e antigen (HBeAg) negative, antibody to HBeAg (anti-HBe) positive, persistently normal alanine aminotransferase (ALT) and aspartate aminotransferase (AST) levels, serum HBV-DNA<2000IU/ml, and, whenever available, liver histology with low grade inflammation (less than 4) [1]. The evidence, however, for these stringent criteria is supported by limited data as there are only few longitudinal cohort studies with a clear-cut definition of “inactive HBsAg *Corresponding author. Prof. Erica Villa. Gastroenterology and Liver Clinic, Azienda Ospedaliero-Universitaria, University of Modena and Reggio Emilia, Modena, Via del Pozzo 71, 41124 Italy. Tel.: +390594225308; fax: +390594224363. E-mail address: [email protected] (E. Villa). 1 carrier”. Although several studies on the natural history of patients with chronic HBV infection have been published, a comprehensive analysis is hampered by the different study designs (case–control, cross-sectional, longitudinal) and the heterogeneity of the patient populations relative to the clinical setting (inactive carrier, asymptomatic carrier, chronic hepatitis). Available data have called into question the concept of inactive carrier, and proposals have been made to speak of an “inactive carrier state” rather than an “inactive carrier”, implying a possible reversion of the condition [2]. A careful understanding of the clinical features and outcome of the inactive HBsAg carrier is relevant for the management of chronic HBV infection. For this purpose this article will review the literature regarding liver function, viral replication, liver histology and clinical course in an attempt to better define the features of the inactive HBsAg carrier state and to evaluate differences in prognosis according to clinical settings (inactive carrier state or chronic hepatitis). 1. Liver function Ø L’associazionedelsolodosaggiodelletransaminasiedell’HBVDNApuò portareadunaperditadicircail10%dimalameepaXcheistologicamente significaXve A major bias influencing the results of studies on patients with chronic HBV infection is the cross-sectional, instead These authors contributed equally. © 2010 Editrice Gastroenterologica Italiana S.r.l. Published by Elsevier Ltd. All rights reserved. DeFranchisR,MeucciG,VecchiM,etal.ThenaturalhistoryofasymptomaXchepaXXsBsurfaceanXgencarriers.AnnInternMed 1993;118:191−4. VelascoM,González-CerónM,delaFuenteC,etal.ClinicalandpathologicalstudyofasymptomaXcHBsAgcarriersinChile.Gut1978 MarXnot-PeignouxM,BoyerN,ColombatM,etal.SerumhepaXXsBvirusDNAlevelsandliverhistologyininacXveHBsAgcarriers.JHepatol 2002;36:543−6. Liver International ISSN 1478-3223 REVIEW ARTICLE The prognosis and management of inactive HBV carriers ! 2, Glenda Grossi1 and Pietro Lampertico1 Federica Invernizzi1, Mauro Vigano 1 ‘A. M. and A. Migliavacca’ Center for Liver Disease, Division of Gastroenterology and Hepatology Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico, Universit! a di Milano, Milano, Italy 2 Hepatology Unit, Ospedale San Giuseppe, Universit! a degli Studi di Milano, Milano, Italy DOI: 10.1111/liv.13006 Abstract Patients with chronic hepatitis B virus (HBV) infection lacking the serum hepatitis B e antigen (HBeAg) and with antibodies against HBeAg (anti-HBe), are the prevalent subgroup of HBV carriers worldwide. The prognosis of these patients is different Ø UXlizzodellatransientelastography(TE)perdiscriminareICdaHBsAgnegaXveCHB from inactive carriers (ICs), who are characterized by persistently normal serum alanine aminotransferase (ALT) and low (<2000 IU/ml) serum HBV DNA levels, a serological profile that may also be intermittently observed in patients with HBeAgnegative chronic hepatitis. This is why a confirmed diagnosis of IC requires quarterly ALT and HBV DNA measurements for at least 1 year, while a single-point detection of combined HBsAg <1000 IU/ml and HBV DNA <2000 IU/ml has a robust predictive value for the diagnosis of IC. Characteristically, ICs have minimal or no histological lesions of the liver corresponding to liver stiffness values on Fibroscan of <5 kPa. Antiviral treatment is not indicated in ICs since the prognosis for the progression of liver disease is favourable if there are no cofactors of liver damage such as alcohol abuse, excess weight or co-infection with the hepatitis C virus or delta virus. Moreover, spontaneous HBsAg loss frequently occurs (1–1.9% per year) in these patients while the development of hepatocellular carcinoma (HCC) is rare, at least in Caucasian patients. However, an emerging issue reinforcing the need for clinical surveillance of ICs is the risk of HBV reactivation in patients who undergo immunosuppressive therapy without receiving appropriate antiviral prophylaxis. After diagnosis, management of ICs includes monitoring of ALT and HBV DNA every 12 months with periodic measurement of serum HBsAg levels to identify viral clearance. Ø NegliIcslasXffnessmediacalcolataneglistudièdi5KPa(limitesuperioredi7.9Kpa) Ø L’evidenzadipiùelevaXdisXffnessinpazienteconsoppressioneviraleenormaliAlt puòindicareundannoepaXcosecondarioadaltracausa,suggerendounpiùabento monitoraggiool’esecuzionediunabiopsiaepaXca Keywords antiviral treatment – HCC – hepatitis B virus – inactive carriers – natural history – prophylaxis Olivieri from patients with HBeAg-negative 2008 4.3+/-1KPa hepatitis because of the different Maimone 2009 4.83+/-1KPa Castera 2011 4.8+/-2KPa An estimated 340 million individuals are chronically infected with the hepatitis B virus (HBV) with a risk of developing end-stage liver complications (1–3). Patients with antibodies against the hepatitis B e antigen (antiHBe) are the largest subgroup of HBV carriers worldwide, and characterized by a broad spectrum of clinical conditions ranging from the inactive carrier (ICs) state to chronic active hepatitis progressing to liver complications such as cirrhosis, liver failure and hepatocellular carcinoma (HCC) (2–4). ICs must be distinguished chronic active prognosis and management of these groups, with a substantial risk of cirrhosis, clinical decompensation and HCC in the latter (4, 5). The distinctive virological feature of patients with chronic active HBV is infection with HBV variants that cannot produce HBeAg because of the presence of precore or basal core promoter mutations. The livers of these patients show persistent necroinflammation characterized by fluctuations of both viral replication and QualeèilrischiodiprogressionedimalamaesviluppoHCC? May 2010 LONG-TERM HEALTH OUTCOMES OF INACTIVE HBV CARR Natural history of chronic hepatitis B C-J Chen and H-I Yang 89 293 individuals invited to participate in 1991–1992 23 820 enrolled in the cohort 4155 HBsAg (+) 19 665 HBsAg (–) 3653 HBV DNA available and anti-HCV (–) 565 HBeAg (+) 3088 HBeAg (–) 2780 HBV DNA detectable 873 HBV DNA undetectable 1520 genotype B 801 genotype C 1619 HBV DNA 84 genotype B+C 104 copies/mL 543 precore 1896 wild-type 705 BCP 1762/2764 wild-type Supplementary Figure 1. Flow of participants 750 precore 1896 mutant 578chart BCP 1762/2764 mutant in the inactive hepatitis B virus carrier and control subcohorts. ALT, alanine aminotransferase; HBsAg, hepatitis B surface antigen; HCV, hepatitis C virus. 30 1932inacXvecarriers(HBsAg+, HBVDNA<10,000copie/mL; ALT<45) Followupsemestralecon ecografiaaddominaleedesami ematochimici 10 000–99 999, Figure 1 Flow of the Risk Evaluation of Viral Load Elevation and Associated Liver Disease/ Cancer-Hepatitis B Virus (REVEAL-HBV) study participants. REVEALHBV 100 000–999 999, 1 000 000–99 999 999, Ø IncidenzaHCCinacXve carriers:64/100000 Ø Incidenzacasicontrollo 15/100000 Ø NondifferenzasignificaXva interminidiincidenzatrai portatoriinamviconvalori diHBVDNAnonrilevabili rispeboaipazienXcon HBV<2000UI/ml CLINICAL–LIVER, Figure 1. Cumulative hazards of progression to hepatocellular carcinoma in the inactive hepatitis B virus (HBV) carrier and control subcohorts. 95% CI: 0.6!4.5). For inactive HBV carriers with undetectable baseline serum HBV DNA, an alcohol drinking A total of 1775 (91.9%) inactive HBV carriers, including 92.2% (772 of 837) and 91.6% (1003 of 1095) of those FATTORIDIRISCHIOHCCNEIPORTATORIINATTIVI Table 2. Multivariate-Adjusted Hazard Ratios (95% Confidence Intervals) of the Risk Predictors for Newly Developed Hepatocellular Carcinoma Included in the Cox Regression Models All participants (n " 20,069) Variable Cohort Control Inactive HBV carrier Undetectable HBV DNA HBV DNA 300!10,000 copies/mL Age (increment by every decade) Male sex (vs female) ALT (high-normal vs low-normal) Alcohol drinking habit (ever vs never) Cigarette smoking habit (ever vs never) Hazard ratio (95% CI) 1.0 (referent) 3.5a 5.7a 2.7 1.3 2.2 2.4 1.7 (1.5!8.3) (2.8!11.5) (1.9!3.8) (0.6!2.9) (1.2!4.1) (1.3!4.7) (0.8!3.5) Inactive HBV carriers (n " 1932) P value Hazard ratio (95% CI) P value 1.0 1.6 2.6 2.9 0.5 5.0 0.5 (referent) (0.6!4.5) (1.4!4.6) (0.7!11.8) (0.1!2.3) (1.6!15.6) (0.2!1.6) .362 .002 .133 .374 .005 .245 — .005 #.001 #.001 .545 .011 .008 .151 ALT, alanine aminotransferase; CI, confidence interval; HBV, hepatitis B virus. " .349 for the difference between serum HBV DNA 300-10,000 copies/mL vs undetectable. aP CarriersofInac:veHepa::sBVirusAreS:llatRiskforHepatocellularCarcinomaandLiver-RelatedDeath.Jin-deChenetal. GASTROENTEROLOGY2010;138:1747–1754 liver) car- CLINICAL–LIVER, PANCREAS, AND BILIARY TRACT Liverrelatedmortality 44/100000vs21/100000 FATTORIDIRISCHIODI“LIVERRELATEDDEATH”NEIPORTATORIINATTIVI Table 3. Multivariate-Adjusted Hazard Ratios (95% Confidence Intervals) of the Risk Predictors for Liver-Related Death Included in the Cox Regression Models All participants (n ! 20,069) Variable Cohort Control Inactive HBV carrier Undetectable HBV DNA HBV DNA 300-10,000 copies/mL Age (increment by every decade) Male sex (vs female) ALT (high-normal vs low-normal) Alcohol drinking habit (ever vs never) Cigarette smoking habit (ever vs never) Hazard ratio (95% CI) Inactive HBV carriers (n ! 1932) P value 1.0 (referent) — 2.4a 1.9a 2.3 1.8 1.9 2.1 1.7 (1.0"5.5) (0.7"4.7) (1.7"3.2) (0.9"4.0) (1.1"3.4) (1.2"3.8) (0.9"3.3) .048 .188 $.001 .120 .025 .013 .094 Hazard ratio (95% CI) P value 1.0 0.8 2.6 1.7 0.3 5.8 1.2 (referent) (0.2"2.7) (1.2"5.3) (0.3"10.9) (0.04"2.7) (1.5"21.9) (0.3"4.6) .733 .011 .552 .307 .010 .837 ALT, alanine aminotransferase; CI, confidence interval; HBV, hepatitis B virus. aP ! .695 for the difference between serum HBV DNA 300"10,000 copies/mL vs undetectable. hepatocellular carcinoma was the most striking risk pre- death for inactive HBV carriers through a communitydictor for liver-related death (HRa ! 611; 95% CI: based long-term follow-up study. As antiviral therapy for CarriersofInac:veHepa::sBVirusAreS:llatRiskforHepatocellularCarcinomaandLiver-RelatedDeath.Jin-deChenetal. 325"1151). Older age was the only significant risk pre- chronic hepatitis B was not reimbursed by national GASTROENTEROLOGY2010;138:1747–1754 skTable Predictors for Newly Developed 3. Multivariate-Adjusted Hazard Ratios (95% Confidence Intervals) of the Risk Predictors for Liver-Related Death Liverrelateddeath Included inHCC the Cox Regression Models Inactive HBV carriers (n " 1932) Hazard ratio Variable (95% CI) Cohort Control Inactive HBV carrier 1.0 (referent) Undetectable HBV DNA 1.6 (0.6!4.5) HBV DNA 300-10,000 copies/mL 2.6 (1.4!4.6) Age (increment by every decade) 2.9 (0.7!11.8) Male sex (vs female) 0.5 (0.1!2.3) ALT (high-normal vs low-normal) 5.0 (1.6!15.6) Alcohol drinking habit (ever vs never) 0.5 (0.2!1.6) Cigarette smoking habit (ever vs never) HBVDNA All participants (n ! 20,069) P value Hazard ratio Inactive HBV carriers (n ! 1932) neg (95% CI) P value Hazard ratio (95% CI) P value 1.0 0.8 2.6 1.7 0.3 5.8 1.2 (referent) (0.2"2.7) (1.2"5.3) (0.3"10.9) (0.04"2.7) (1.5"21.9) (0.3"4.6) .733 .011 .552 .307 .010 .837 HBVDNA<2000UI/ml .362 .002 .133 .374 .005 .245 1.0 (referent) 2.4a 1.9a 2.3 1.8 1.9 2.1 1.7 (1.0"5.5) (0.7"4.7) (1.7"3.2) Alcool (0.9"4.0) (1.1"3.4) (1.2"3.8) (0.9"3.3) ETA’ — .048 .188 $.001 .120 .025 .013 .094 ALT, alanine aminotransferase; CI, confidence interval; HBV, hepatitis B virus. ble. aP ! .695 for the difference between serum HBV DNA 300"10,000 copies/mL vs undetectable. hepatocellular carcinoma was the most striking risk pre- death for inactive HBV carriers through a communitydictor for liver-related death (HRa ! 611; 95% CI: based long-term follow-up study. As antiviral therapy for 325"1151). Older age was the only significant risk pre- chronic hepatitis B was not reimbursed by national dictor independent of hepatocellular carcinoma for con- health insurance in Taiwan until 2003, the participants trols, but not for inactive HBV carriers. Subcohort was no in this study had not received any antiviral treatment. longer an independent risk predictor for liver-related CarriersofInac:veHepa::sBVirusAreS:llatRiskforHepatocellularCarcinomaandLiver-RelatedDeath.Jin-deChenetal. Few studies in the past have addressed the long-term GASTROENTEROLOGY2010;138:1747–1754 mortality when newly developed hepatocellular carci- follow-up of inactive HBV carriers. Gigi and colleagues10 Ø IlrischiodisviluppodiHCCperiportatoriinamviHBV èmaggiorepercolorochepresentanounHBVDNA misurabilerispeboacolorocherisultanoHBVDNA NEGATIVI.TaledifferenzaNONèsignificaXva Ø Inconsiderazionedelleevidenzeemerselasogliadi HBVDNAconsiderataaffidabilenell’individuareil portatoreinamvoèdi10000copie/ml Ø L’etàèunimportantefaboredirischioperlosviluppo diHCC Ø IpazienXcheraggiungonoprimalostadiodiportatore inamvohannounaminorpossibilitàdiavereuna progressionedimalama Tassoannualediincidenza HCC0.06% Tassoannualediincidenzadi “liverrelateddeath”0.04% Ø Valorinormali-alXdiALTnoncorrelanoconilrischiodi HCCedimorte“fegatocorrelata” Ø LosviluppodiHCCèilpiùimportantefaboredirischio dimortalitàperiportatoriinamvi CarriersofInac:veHepa::sBVirusAreS:llatRiskforHepatocellularCarcinomaandLiver-RelatedDeath.Jin-deChenetal. GASTROENTEROLOGY2010;138:1747–1754 Natural history of chronic hepatitis B C-J Chen and H-I Yang Milestone 1: Start of decline of HBV DNA level HBV DNA >108 copies/mL HBeAg/Anti-HBe status Milestone 2: HBeAg/ Anti-HBe seroconversion Milestone 3: HBV DNA decreased to undetectable level Milestone 4: Clearance of HBsAg HBV DNA level HBeAg+, Anti-HBe- HBeAg-, Anti-HBe+ Undetectable level of HBV DNA HBsAg+ HBsAg status HBsAg- ΔT ALT level Immune tolerance Immune clearance Residual Figure 4 Natural history of chronic hepatitis B based on the Risk Evaluation of Viral Load Elevation and Associated Liver Disease/Cancer-Hepatitis B Virus (REVEAL-HBV) Study findings. MA… Ø PazienX30-65anni Ø Mancatodosaggioseriatodell’HBVDNA Ø NonconsideraXigenoXpiHBV CarriersofInac:veHepa::sBVirusAreS:llatRiskforHepatocellularCarcinomaandLiver-RelatedDeath.Jin-deChenetal. GASTROENTEROLOGY2010;138:1747–1754 Digestive and Liver Disease 46 (2014) 427–432 Contents lists available at ScienceDirect Digestive and Liver Disease journal homepage: www.elsevier.com/locate/dld Liver, Pancreas and Biliary Tract Progression to cirrhosis, hepatocellular carcinoma and liver-related mortality in chronic hepatitis B patients in Italy Donatella Ieluzzi a,b,1 , Loredana Covolo c,1 , Francesco Donato c , Giovanna Fattovich a,d,∗ a Division of Gastroenterology and Endoscopy, Azienda Ospedaliera Universitaria Integrata, Verona, Italy Department of Surgery, University of Verona, Verona, Italy Institute of Hygiene, Epidemiology and Public Health, University of Brescia, Brescia, Italy d Department of Medicine, University of Verona, Verona, Italy b c a r t i c l e i n f o a b s t r a c t Ø 105pazienX(F25,M80)etàmedia30anni Article history: Received 11 July 2013 Accepted 10 January 2014 Available online 16 February 2014 Keywords: Chronic hepatitis B Cirrhosis Hepatocellular carcinoma Liver-related mortality Natural history Background: The natural history of chronic hepatitis B is variable. We evaluated some of the risk factors for cirrhosis, hepatocellular carcinoma and liver-related mortality in Italian patients with chronic hepatitis B. Methods: A cohort of 105 untreated patients with chronic hepatitis B without cirrhosis at diagnosis was followed prospectively for a mean period of 23 years. Clinical, histological and ultrasound examinations, biochemical and virological tests, and causes of death were analyzed. Results: Forty-two (40%) patients became inactive carriers and 63 (60%) showed persistent alanine aminotransferase elevation: 13 (13%) associated with HBeAg persistence, 35 (33%) with detectable serum HBV-DNA but HBeAg-negative, 11 (10%) with concurrent virus infection and 4 (4%) with non-alcoholic fatty liver disease. Cirrhosis incidence was 1.56/100 person-years. Older age and sustained HBV replication predicted cirrhosis occurrence independently. Hepatocellular carcinoma incidence was 2.1/100 person-years in patients who developed cirrhosis and 0.06 in those who did not. Cirrhosis occurrence was associated with an increased risk of hepatocellular carcinoma (hazard ratio 20.4, 95% confidence interval 2.54–167.5) and liver-related death (16.5, 2.0–138.8). Conclusions: In Italian patients with chronic hepatitis B cirrhosis strongly predicts hepatocellular carcinoma occurrence and disease-related mortality, thus indicating that early antiviral treatment should be instituted before cirrhosis occurrence. © 2014 Editrice Gastroenterologica Italiana S.r.l. Published by Elsevier Ltd. All rights reserved. Ø Followupsemestrale(ecografia+esamiematochimici)peri pazienXconepaXteamvaeannualepergliinacXvecarriers (HBeAgnegaXvi,HBVDNA<limitesogliadirilievo) 1. Introduction Chronic hepatitis B virus (HBV) infection is a worldwide public health problem, accounting for approximately half a million deaths each year. The natural history of chronic HBV infection varies from one individual to another and from one geographical area to another, possibly due to differences in viral, host and environmental factors [1,2]. Several studies have described the characteristics, clinical course and risk of liver-related complications of chronic HBV infection in the Mediterranean area, but a comparison of the results is hampered by different study design, the small number of patients recruited, short follow-up and the heterogeneity of the patient population in relation to the severity of chronic liver disease at diagnosis. A careful understanding of the clinical course and factors influencing progression of chronic hepatitis B (CHB) diagnosed at an early stage would be particularly important for monitoring and taking evidence-based decisions on patient care in clinical practice. At present many subjects with CHB undergo anti-viral treatment, the standard of care consisting of life-long oral nucleos(t)ide analogues (NAs), which have been found to suppress circulating viral loads efficiently and can lead to regression of fibrosis [3]. At present few data on the natural history of CHB in Western countries are available for evaluating the magnitude of the clinical benefit of antiviral therapy. In this study we aimed to investigate the natural history of CHB, with emphasis on the risk of, and factors related to, clinical progression to cirrhosis, hepatocellular carcinoma (HCC) and liver-related mortality in a cohort of Caucasian patients in Italy with CHB, without histological and clinical evidence of cirrhosis at diagnosis. Ø Duratamediadelfollowup:23.2anni(28.8perIC*) ∗ Corresponding author at: Unità Operativa Complessa di Gastroenterologia ed Endoscopia Digestiva dO, Dipartimento di Medicina, Azienda Ospedaliera Universitaria Integrata Verona, Piazzale A. Stefani n. 1, 37126 Verona, Italy. Tel.: +39 045 8127257; fax: +39 045 81272014. E-mail address: [email protected] (G. Fattovich). *InacXvecarrier 1 105pazienX HBsAg-;AnXHBsAg+ 2duranteHBeAg+prima dellasieroconversionead anXHBe 2HBeAgnegaXviprima dellanormalizzazionedelle transaminasi 38 NESSUNODEIPORTATORIINATTIVIHASVILUPPATOHCCo èdecedutopercausecorrelateaproblemaXcheepaXche *NB:difabopazienXgiàarrivaticirroXciprimadellafasediianmvità LiverInternaXonal(2016) IncidenceofhepatocellularcarcinomainuntreatedsubjectswithchronichepaXXsB:a systemaXcreviewandmeta-analysis ElenaRaffem1,GiovannaFabovich2andFrancescoDonato Ø StudilongitudinalietrialsrandomizzaXcontrollaX(pz>18anni) Ø SoggemcondiagnosidiepaXtecronica,cirrosi,portatoriinamvi(ALT costantementenormali,HBeAgnegaXvi,anXHBeAgposiXvi), portatoriasintomaXci Ø StudidacuirilevaredaXidoneialcalcolodeitassidiincidenza Ø 66studi,347859pazienX Ø IncidenzaHCC Outcomemeasure Ø RischiorelaXvoHCCperareageografica,gradodimalama,genere,età * R I S U L T A T I *x100/anno EUROPA Portatore asintomaXco 0.07 N.AMERICA ASIA 0.19 0.42 Portatoreinamvo 0.03 0.17 0.06 Ø L’incidenzadiHCCaumentainmanieracrescentedalportatoreinamvoalportatore EpaXtecronica 0.12 0.48 0.49 asintomaXco,conepaXtecronicaeconcirrosicompensata Cirrosi 2.03 2.89 3.37 Ø compensata Correlazioneconetàmanonconareageografica Ø Incrementodiduevoltedelrischioperunconsumoalcolicodi60g/die Raffetti et al. Incidence of HCC in subjects with chronic HBV Ø MaggiorrischiodisviluppodiHCCpergenoXpoCrispeboalB Ø HBsAg>1000IU/ml Fig. 3. Five-year cumulative HCC risk according to macro-area and liver disease status (Europe and East Asia). Abbreviation: HCC, hepatocellular carcinoma. 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Reac:va:onofhepa::sB 1998;49:385–8. a^ertransplanta:onopera:ons.Lancet.1977;68:105–12 Hepa::sBvirusandhepa::sB-relatedviralinfec:onin Reac:va:onofhepa::sBvirusinHBsAgnega:ve renaltransplantrecipients.Gastroenterology1988;94:151–156 pa:entswithmul:plemyeloma:twocasereport HepaXXsBreacXvaXoninthesemngofchemotherapyand immunosuppression-prevenXonisbeberthancure YoshidaetalIntJHematology.2010Jun,91(5):844-9 WJH2015 Tra)amentodell’epa2teBcronica:raccomandazionidaunworkshopitaliano- Diges2veandLiverDisease40(2008)603-617 La profilassi è indicata: Nei portatori inattivi di HBsAg sottoposti a chemioterapia antitumorale o a trattamento immunosoppressivo ad alto rischio: anti-TNF, anti CD20, anti CD56 a steroidi a dosaggi medio elevati (>7.5mg/die) per periodi prolungati, ciclofosfamide, metotrexato, leflunomide, ciclosporina, tacrolimus, azatioprina, micofenolato I portatori cronici inattivi e i pazienti HBsAg negativi e anti-HBc-positivi devono essere sottoposti a profilassi fin dall’inizio del trattamento immunosoppressivo o, se possibile, a partire da 2-4 settimane prima che cominci la somministrazione dei farmaci immunosoppressori (CIII) La profilassi mirata deve essere iniziata al momento della riattivazione della malattia (HBV-DNA >2000UI/ml) nei portatori di HBsAg, e immediatamente dopo la sieroconversione nei pazienti che diventano HBsAg positivi (CIII) Risk stratification for HBV reactivation Drugs High Disease Oncohematology and BMT IBD Medium TNFa-inhibitors Other biological DMARDs Leflunomide Methotrexate Cyclophosfamide HBsAg+ Combination therapies Medium/high-dose prednisone (>7.5 mg/die) Null Low Rheum RISK HIV HBsAg+ anti-HBc+ Rituximab combined therapy SOTs Virus Calcineurin inhibitors Azathioprine 6-mercaptopurine Low-dose prednisone (<7.5 mg/die) Sulfasalazine Hydroxychlorochine Marzano A et al., DLD 2007 (2011) RischiodiriamvazioneneipazienX immunocompromessi BonemarroworstemcelltransplantaXon HBsAg-posiXve HBsAg-negaXve,anX-HBc-posiXve 32–50% upto50% AnX-CD20monoclonalanXbodies(rituximab) HBsAg-posiXve HBsAg-negaXve,anX-HBc-posiXve 50-80% 18% SolidorgantransplantaXon HBsAg-posiXve HBsAg-negaXve,anX-HBc-posiXve 50-90% 0.9-5% 39-41% 3% 39% 5% Systemiccancerchemotherapy HBsAg-posiXve HBsAg-negaXve,anX-HBc-posiXve TNF-alfaantagonists HBsAg-posiXve HBsAg-negaXve,anX-HBc-posiXve Hwang and Lok Nat Rev Gastroenterol 2013 Hepat Mon. 2016 April; 16(4):e35810. doi: 10.5812/hepatmon.35810. Review Article Published online 2016 March 26. Hepatitis B Reactivation During Immunosuppressive Therapy or Cancer Chemotherapy, Management, and Prevention: A Comprehensive Review Soheil Tavakolpour,1 Seyed Moayed Alavian,1,* and Shahnaz Sali2 1 Baqiyatallah Research Center for Gastroenterology and Liver Diseases, Baqyiatallah University of Medical Sciences, Tehran, IR Iran Infectious Diseases and Tropical Medicine Research Center, Shahid Beheshti University of Medical Sciences, Tehran, IR Iran 2 * Corresponding author: Seyed Moayed Alavian, Baqiyatallah Research Center for Gastroenterology and Liver Diseases, Baqyiatallah University of Medical Sciences, Tehran, IR Iran. Tel/Fax: +98-2181264070, E-mail: [email protected] Tavakolpour Received 2015 December 26; Revised 2016 January 17; Accepted 2016 January 27. Ø Lalamivudinapuòessereconsideratacomelapiùcomunescelta Abstract perlaprofilassineipazienXHBV avoided in cases under HSCT, even in the presence of complete remission (52, 61). Thus, prophylaxis longer than 24 months was recommended for these patients (54). On the issue of HSCT, several studies have reported HBVr after more than Context: Due to the close relationship between the immune system and the hepatitis B virus (HBV) replication, it is essential to 12 months, reaching a peak of 91 months (27, 54, 56, 61). monitor patients with current or past HBV infection under any type of immunosuppression. Cancer chemotherapy, immunosupConsidering the high resistance to lamivudine (LAM), in are addition pressive therapies in autoimmune diseases, and immunosuppression in solid organ and stem cell transplant recipients the to various reported cases with a risk of reactivation following more than 6 months from the cessation major reasons for hepatitis B virus reactivation (HBVr). In this review, the challenges associated with HBVr are discussed according of immunosuppressive therapy or chemotherapy, it is recommended to the latest studies and guidelines. We also discuss the role of treatments with different risks, including anti-CD20 agents, tumor that referenced HBV antiviral agents, such as entecavir (ETV) and TNV should be used in therapies that necrosis factor-alpha (TNF- ) inhibitors, and other common immunosuppressive agents in various conditions. need long-term prophylaxis. Recommendations of various bodies associated with HBVr are shown in Table 2. Evidence Acquisition: Through an electronic search of the PubMed, Google Scholar, and Scopus databases, we selected the studies In a meta-analysis, LAM prophylaxis in HBsAg carriers associated with HBVr in different conditions. The most recent recommendations were collected in order to reach a consensus on with breast cancer under chemotherapy was introduced as an effective option in the reduction of HBVr (95). Based on how to manage patients at risk of HBVr. a systematic review, LAM prophylaxis had appeared as an Results: It was found that the positive hepatitis B surface antigen (HBsAg), the high baseline HBV DNA level, the positive effective hepatitis option in the reduction of HBVr in HBsAg-positive B virus e antigen (HBeAg), and an absent or low hepatitis B surface antibody (HBsAb) titer prior to starting treatment are lymphoma the most patients undergoing chemotherapy. After that, preventing reactivation caused a decrease in the disrupimportant viral risk factors. Furthermore, rituximab, anthracycline, and different types of TNF- inhibitors were identified as the tion of the chemotherapy (96). high-risk therapies. By analyzing the efficiency of prophylaxis on the prevention of HBVr, it was concluded that those with a high In risk another report, anti-HBc-positive patients under immunosuppressive therapy were included (97). These paof antiviral resistance should not be used in long-term immunosuppressants. Receiving HBV antiviral agents at the commencement tients suffered from various autoimmune diseases. Among of immunosuppressant therapy or chemotherapy was demonstrated to be effective in decreasing the risk of HBVr. Prophylaxis could those who had received antiviral therapy, none indicated in HBV DNA levels, while 11.5% of the control group also be initiated before the start of therapy. For most immune suppressive regimes, antiviral therapy should be kept up foraatrise least 6 did experience an increase. ALT elevation was significantly months after the cessation of immunosuppressive drugs. However, the optimal time of prophylaxis keeping should be increased lower in in the antiviral prophylactic group. Additionally, one in the control group showed reverse seroconvercases associated with rituximab or hematopoietic stem cell transplants. According to the latest studies and guidelines frompatient different sion. In contrast, no reverse seroconversion was observed bodies, recommendations regarding screening, monitoring, and management of HBVr are outlined. in the prophylactic group. LAM can be considered as the most common prophyConclusions: Identification of patients at the risk of HBVr before immunosuppressive therapy is an undeniable part of treatment. laxis in HBV patients. However, there is a considerable risk Starting the antiviral therapy, based on the type of immunosuppressive drugs and the underlying disease, could lead to better manof LAM resistance risk in patients with active HBV. Based on the literature, LAM is an appropriate choice in cases with agement of disease. high viral replication that need short-term treatments. In contrast, it was not recommended for HBsAg carriers with Hepatitis B Virus, Reactivation, Immunosuppression, Rituximab, Prophylaxis detectable HBV DNA levels at baseline, who are candidates for long-term immunosuppressive therapy. Accordingly, it can be concluded that LAM prophylaxis is a reasonable choice for OBI/resolved HBV patients. LAM is more common in countries with a high preva- Ø Lalamivudinarappresentaunasceltaappropriataneicasicon elevatareplicaviralechenecessitanoditrabamenXdibreve ↵ durata Ø SceltaragionevolenegliOBI Keywords: ↵ La lamivudina si è mostrata efficace nella profilassi della riacutizzazione HBV(0.9% vs 25-85% dei non trattati negli studi retrospettivi e 5% vs 24 negli studi prospettici) 2008 La profilassi con lamivudina è indicata nei pazienti HBsAg+ con HBV DNA < 2000 UI/ml,; nei pazienti con HBV DNA> 2000UI/ml , che devono essere sottoposti a cicli di durata maggiore o ripetuti, è indicato l’utilizzo di analoghi ad alta barriera genetica (tenofovir o entecavir) 2012 La terapia profilattica antivirale è raccomandata per i portatori di HBV all’ inizio della terapia ed almeno sei mesi dopo, i farmaci ad alta barriera genetica sono raccomandati quando previsti trattamenti di lunga durata o quando vengono utilizzati farmaci ad alto rischio come il Rituximab 2016 La profilassi è indicata nei pazienti HBsAg che devono essere sottoposti a chemioterapia o a regimi di immunosoppressione sia durante che per 6-12 mesi dopo la fine del trattamento 2016 with normal baseline ALT has been reported to be higher during the first year (15–20%) [12,14] and declines after 3 years of follow-up [9,14,15], frequent monitoring during the first 1–3 years is critical in determining whether a patient has PNALT. Besides the frequency of determinations and duration of follow-up, definition of PNALT should include standardized criteria for the upper limit of normal (ULN) of ALT. Some investigators have suggested that lower cut-offs than the traditional values defined by most diagnostic laboratories (around 40 IU/L) should be used. These suggestions were derived from studies based on single ALT values in Italian blood donors [16], the associations between normal ALT and increased rates of liver related deaths in Koreans applying for life insurance [17], and high serum HBV DNA levels or increased risks of complications of liver disease tional cut-offs for the ULN of ALT values in patients with HBeAg-negative chronic HBV infection. Severe liver histological lesions were rare among HBeAg-negative patients with truly PNALT. In the four studies with good or acceptable definitions of PNALT [7,8,11,12], more than a minimal (always mild) inflammatory activity was detected in only 3% and more than mild fibrosis always with minimal inflammatory activity in only 5% (moderate fibrosis: 4.5%, severe fibrosis: 0.5%, cirrhosis: 0%) of 215 patients with serum HBV DNA 620,000 IU/ ml. The prevalence of mild inflammatory activity and moderate fibrosis was 7% and 10% among patients with HBV DNA levels between 2000 and 20,000 IU/ml and 1.4% and 1% among those with HBV DNA levels <2000 IU/ml. Similar mild histological findings in HBeAg-negative patients with HBV DNA 620,000 IU/ml Qualefollowup? Review Follow-up and indications for liver biopsy in HBeAg-negative chronic hepatitis B virus infection with persistently normal ALT: A systematic review George V. Papatheodoridis1,⇑, Spilios Manolakopoulos1, Yun-Fan Liaw2, Anna Lok3 1 HBeAg-negative patient with normal ALT at baseline 2nd Department of Internal Medicine, Athens University Medical School, ‘‘Hippokration’’ General Hospital of Athens, Greece; 2Liver Research Unit, Chang Gung Memorial Hospital, Chang Gung University College of Medicine, Taipei, Taiwan; 3Division of Gastroenterology and Hepatology, University of Michigan Health System, Ann Arbor, MI, USA ALT every 3-4 mo for one year + serum HBV DNA determination Introduction Background & Aims: The adequacy of monitoring HBeAg-negaALT >ULN and/or Persistently ALT <ULN and Persistently ALT <ULN and tive patients based on ALT activity is controversial and current HBV DNA IU/ml HBVnorDNA 2000-20,000 IU/mlapproximately 400 million people HBV DNA <2000 IU/ml Worldwide, have chronic infecguidelines favor liver>20,000 biopsy in HBeAg-negative cases with tion with hepatitis B virus (HBV), which usually runs a variable mal ALT and HBV DNA >2000 IU/ml. We systematically reviewed course lasting for decades and often the entire life, with outcomes all the available histological data on HBeAg-negative patients ALT every 3-4 mo, ranging from asymptomatic carrier state to cirrhosis, liver failure, with persistently normal ALT (PNALT) to determine the prevaHBV DNA every year and and hepatocellular carcinoma (HCC) [1–4].ALT Theevery natural history of lence of significant liver disease and its associating factors. Liver biopsy 6 mo* transient elastography every year chronic Methods: Literature search to identify studies with adult HBeAgfor two more yearsHBV infection has been divided into different phases, based on hepatitis B e antigen (HBeAg) status, serum HBV DNA levels, and negative patients who had PNALT as defined by the authors, a alanine aminotransferase (ALT) activity [1,2]. minimum follow-up of 1 year and histological data. Traditional In the HBeAg-negative phases, the majority of patients remain cut-off values of normal ALT were used in all studies. The definiALT <ULN and DNA 2000-20,000 IU/ml low-replicative state (inactive HBV carrier state). in an inactive, tions of PNALT were considered as acceptable or good HBV if there Inactive carriers are negative for HBeAg; and have persistently were P3 ALT determinations at unspecified intervals during 6– normal ALT (PNALT), low or undetectable serum HBV DNA levels, 12 months or predefined intervals during P12-month periods, ALT every 6 mo, or no necroinflammation and generally minimal to mild minimal respectively. HBV DNA every year and fibrosis on liver biopsy. A proportion of HBeAg-negative patients, Results: Six studies including 335 patients met our inclusion critransient everymay yearprogress to an immune active phase (HBeAg-neghowever, teria. Of these, four studies with 246 patients had good or accept-elastography ative chronic hepatitis B) with persistently or transiently elevated able definitions of PNALT. In the latter four studies, more than ALT and high serum HBV DNA levels [1–3]. minimal (usually mild) necro-inflammatory activity was ALT >ULN and/or, Theoretically, serum levels of ALT, an enzyme that is released observed in 10% and more than mild fibrosis in 8% of all patients HBV DNA >20,000 IU/ml and/or from hepatocytes liver should reflect the degree of (moderate fibrosis: 7%, severe fibrosis: 1%, cirrhosis: 0%), and in liverduring stiffness >9 injury, kPa liver damage [5]. However, several reports have suggested that 3% and 5% of patients with HBV DNA 620,000 IU/ml, respectively. patients with chronic HBV infection who are HBeAg negative Conclusions: Histologically significant liver disease is rare in and have normal ALT may have high serum HBV DNA levels HBeAg-negative patients with PNALT based on stringent criteria Liver biopsy and significant histological liver damage [6,7]. Such cases have and serum HBV DNA 620,000 IU/ml. Such cases can be considcreated debates and clinical dilemmas regarding the adequacy ered as true inactive HBV carriers, who require neither liver Fig. 1. Clinical algorithm for the optimal follow-up and management of patients with HBeAg-negative chronic HBV infection and normal alanine aminotransferase of monitoring HBeAg-negative patients based on ALT activity biopsy(ALT) noractivity immediate therapy but continued follow-up. at baseline. All patients should have ALT and HBV DNA monitoring every 3–4 months during the first year. Patients with ALT persistently below ULN and HBV ⁄ only, the need liver biopsy, and indication for treatment ! 2012 European Association for the the Liver. Published DNA <2000 IU/ml during the first yearStudy may beofmonitored with ALT every 6 months. Periodical HBVfor DNA measurements may the be also helpful, although the optimal JV.Papatheodoridisetal.JournalofHepatology2012vol.57j196–202 [3]. Welimit systematically reviewed all the available histological data by Elsevier B.V. All rights reserved. frequency of HBV DNA monitoring is unclear. Patients with ALT persistently below the upper of normal (<ULN) and HBV DNA 2000–20,000 IU/ml during the first year Review Grazieperl’abenzione IngeneraleloscreeningèconsideratocosteffecXvequandol’incidenza annualediHCCeccedelo0.2%neinoncirroXciel’1.5%neipazienXcirroXci