Autoimmune Hepatitis–PBC Overlap Syndrome: A Simplified Scoring System May Assist in the Diagnosis

Neuhauser, Matthias; Bjõrnsson, Einar; Treeprasertsuk, Sombat; Enders, Felicity; Silveira, Marina G.; Talwalkar, Jayant A.; Lindor, Keith D.

doi:10.1038/ajg.2009.616

Cited by 102 publications

(78 citation statements)

References 19 publications

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“…Az AIH/PBC overlap szindróma kórle-folyása rosszabb, gyorsabb a cirrhosisba, májelégtelen-ségbe való progresszió [27].…”

Section: Aih/pbc Overlapunclassified

“…PBC-s betegekben a PBC/AIH overlap szindróma gyakorisága 2,1% volt a módosított AIHG-pontrendszert alkalmazva [32]. A valószínűleg PBC/AIH gyakorisága PBC-s betegekben 12, illetve 6% volt, attól függően, hogy a módosított IAIHG (International Autoimmune Hepatitis Group) -pontrendszert vagy az egyszerűsített IAIHG-pontrendszert használták [27] -l. később.…”

Section: Aih/pbc Overlapunclassified

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Review of overlap syndromes of autoimmune liver diseases. Difficulties in the diagnosis and treatment

Hagymási

Tulassay

2013

Orvosi Hetilap

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Az overlap szindróma az autoimmun májbetegségek közötti biokémiai, szerológiai, hisztológiai vagy radiológiai átfedéseket, az autoimmun hepatitis és primer biliaris cirrhosis vagy primer szklerotizáló cholangitis együttes fennállását jelenti. Pontos gyakoriságuk nem ismert, 5-20%-os előfordulásról számolnak be. Az overlap szindrómáknak nincs kórjelző diagnosztikus vizsgálata, a klinikai tünetek, biokémiai, szerológiai, radiológiai és hisztológiai vizsgálatok eredményének együttes értékelésén alapul a kórisme. A domináns megjelenésen alapuló, egyénre szabott, urzodezoxikólsavval végzett és/vagy immunszuppresszív (kortikoszteroid-) kezelést a tapasztalatok, illetve retrospektív, nem randomizált vizsgálatok eredményei alapozzák meg. Orv. Hetil., 2013, 154, 923-929. Kulcsszavak: overlap szindróma, kórisme, kezelés, urzodezoxikólsav, immunszuppresszió Review of overlap syndromes of autoimmune liver diseases Diffi culties in the diagnosis and treatmentOverlap syndromes are biochemical, serological, histological and radiological overlaps across the classic autoimmune liver diseases in the presence of autoimmun hepatitis and primary biliary cirrhosis or primary sclerosing cholangitis. The exact prevalence of the disease is not known, but it may vary between 5% and 20%. Because it has no generally accepted diagnostic criteria, clinical signs, biochemical, serological, radiological and histological fi ndings are evaluated together. Treatment depends on the predominant feature of the overlap syndrome; ursodeoxycholic acid and/or immunsuppressive (corticosteroid) treatment are used, based on observations from retrospective, non-randomized studies. Orv. Hetil., 2013, 154, 923-929. Keywords: overlap syndromes, diagnosis, treatment, ursodeoxycholic acid, immunsuppression (Beérkezett: 2013. április 20.; elfogadva: 2013. május 16.) Rövidítések AIH = autoimmun hepatitis; AiSC = autoimmun szklerotizáló cholangitis; Alba = acetylation lowers binding affi nity; AMA = antimitokrondiális antitest; ANA = antinukleáris antitest; ASGP-R = aszialoglikoprotein-receptor; EASL = European Association for the Study of the Liver; ERCP = endoszkópos retrográd cholangiopancreatographia; IAIHG = International Autoimmune Hepatitis Group; IBD = gyulladásos bélbetegség; MRCP = mágneses rezonanciás cholangiopancreatographia; pANCA = perinukleáris antineutrophil cytoplasmaticus antitest; PBC = primer biliaris cirrhosis; PDC-E2 = piruvát-dehidrogenáz komplex E2 alegysége; PSC = primer szklerotizáló cholangitis; SMA = simaizom-ellenes antitest; UDCA = urzodezoxikólsav Az autoimmun hepatitis (AIH), a primer biliaris cirrhosis (PBC), valamint a primer szklerotizáló cholangitis (PSC) nem pontosan ismert immunfolyamatokkal jellemzett májbetegségek, jellegzetes immunológiai, szerológiai és szövettani eltérésekkel. Ha egy időben két

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“…Az AIH/PBC overlap szindróma kórle-folyása rosszabb, gyorsabb a cirrhosisba, májelégtelen-ségbe való progresszió [27].…”

Section: Aih/pbc Overlapunclassified

Review of overlap syndromes of autoimmune liver diseases. Difficulties in the diagnosis and treatment

Hagymási

Tulassay

2013

Orvosi Hetilap

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“…The classic lesion of PSC in the histological study is onionskin or concentric periductular fibrosis, with damage to the ductal epithelium, but it is rarely seen on percutaneous biopsy. The most common findings on a biopsy in early-stage disease are nonspecific [46], fibrosis with inflammation of portal tracts and paucity of normal bile ducts. In addition, in patients with extrahepatic obstruction, proliferation and dilatation of interlobular ducts and an increased number of periportal PMNs can be observed.…”

Section: Primary Sclerosing Cholangitis (Psc)mentioning

confidence: 99%

“…In 1993, the International Autoimmune Hepatitis Group (IAIHG) proposed specific diagnostic criteria, which were revised in 1999. These criteria were made by expert consensus and introduced to allow comparison of studies from different centers [46]. Some of the items were of questionable value which is why in 2008 the IAIHG published a new simplified scoring system for wider applicability in routine clinical practice, based on the data of patients with well-established diagnoses and validated in another group of patients [47].The new score includes autoantibodies, immunoglobulin G, histology, and exclusion of viral hepatitis, allowing a reliable diagnosis of AIH applying simple scores.…”

Section: Autoimmune Hepatitis (Aih)mentioning

confidence: 99%

Rethinking the Role of Liver Biopsy in the Era of Personalized Medicine

Taltavull¹

2012

Liver Biopsy - Indications, Procedures, Results

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“…AIH is also known to be frequently associated with additional autoimmune phenomena (8); however, unlike PBC, the frequency of antiplatelet antibodies among patients with AIH has not been reported. PBC-AIH overlap syndrome is a simultaneous or consecutive association of PBC and AIH (9,10) and, like PBC and AIH, is frequently associated with extrahepatic autoimmune disorders (11); however, the frequency of antiplatelet antibodies has not yet been studied despite occasional case reports of the association with immune thrombocytopenia (ITP) (12)(13)(14)(15).…”

Section: Introductionmentioning

confidence: 99%

Association of Primary Biliary Cirrhosis-autoimmune Hepatitis Overlap Syndrome with Immune Thrombocytopenia and Graves' Disease

et al. 2015

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A 54-year-old woman suffering from pruritus for five years was diagnosed to have Graves' disease and immune thrombocytopenia (ITP) associated with primary biliary cirrhosis (PBC)-autoimmune hepatitis (AIH) overlap syndrome, which was confirmed histologically after a prompt recovery in the platelet count number following steroid therapy. The association between PBC-AIH overlap syndrome and ITP has been rarely reported and the additional association with Graves' disease has not yet been reported. An underlying global derangement of autoimmunity or shared genetic susceptibility was suspected.

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Autoimmune Hepatitis–PBC Overlap Syndrome: A Simplified Scoring System May Assist in the Diagnosis

Cited by 102 publications

References 19 publications

Review of overlap syndromes of autoimmune liver diseases. Difficulties in the diagnosis and treatment

Review of overlap syndromes of autoimmune liver diseases. Difficulties in the diagnosis and treatment

Rethinking the Role of Liver Biopsy in the Era of Personalized Medicine

Association of Primary Biliary Cirrhosis-autoimmune Hepatitis Overlap Syndrome with Immune Thrombocytopenia and Graves' Disease

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