The diagnosis of antimitochondrial antibody-negative primary biliary cholangitis

Özaslan, Ersan; Efe, Cumali; Ozaslan, Nihal Gokbulut

doi:10.1016/j.clinre.2016.06.001

Cited by 31 publications

(32 citation statements)

References 42 publications

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“…After identification of an elevated ALP, assessment should be made to detect the presence or absence of an elevated AMA. AMA is the hallmark antibody of PBC, and is found in over 95% of patients with the disease [12,[19][20][21][22][23][24][25][26][27]. Approximately, 5% of PBC patients will lack a detectable AMA.…”

Section: Diagnosis Of Pbcmentioning

confidence: 99%

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Emerging therapies for PBC

Chascsa

Lindor

2020

J Gastroenterol

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Primary biliary cholangitis is an uncommon cholestatic liver disease predominantly affecting middleaged women. Left untreated, there is a high risk of progression to end-stage liver disease. Few treatment options exist. To date, ursodeoxycholic acid (UDCA) and obeticholic acid (OCA) are the only medical therapies approved for use, other than symptomatic treatments and liver transplantation, the latter of which is reserved for those developing complications of cirrhosis or with intractable pruritus. UDCA improves outcomes, but many patients do not adequately respond. OCA therapy may improve response, but long-term data are limited. New therapies are desperately needed, but evaluation has been limited by the fact that the disease is heterogeneous, hard end points take years to develop, and there are different criteria in use for determining therapeutic response based on surrogate biomarkers. Fibrates appear to be the most promising new therapy and have beneficially affected surrogate end points and are beginning to show improvement in clinical end points.

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Section: Diagnosis Of Pbcmentioning

confidence: 99%

“…The classic histologic finding is a florid duct lesion ( Fig. 1), but it is only present in a minority of histologic samples [26,28]. There is a spectrum of histologic changes.…”

Section: Diagnosis Of Pbcmentioning

confidence: 99%

Emerging therapies for PBC

Chascsa

Lindor

2020

J Gastroenterol

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“…die E2-Untereinheit des Pyruvatdehydrogen-asekomplexes (PDC-E2) bei 80 % der AMA-M2 positiven PBC-Patienten als eigentliches Antigen fungiert. In den 10 % der AMA-negativen PBC finden sich in der Hälfte der Fälle PBC-spezifische ANA vom Typ Anti-sp100 mit dem Immunfluoreszenzmuster der "nuclear dots" oder vom Typ Anti-gp210 mit perinukleärem Immunfluoreszenzmuster [18,30,34,37]. Einzelne PBC gehen ausschließlich mit Autoantikörpern gegen Zentromere einher [37]…”

Section: Primäre Biliäre Cholangitisunclassified

Autoimmune Lebererkrankungen

Fischer

Goltz

2020

Pathologe

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Die Indikation zur Leberbiopsie bei entzündlichen Lebererkrankungen hat sich in den letzten Jahrzehnten erheblich gewandelt. Serologische Untersuchungen erlauben es, Hepatitiden durch hepatotrope Viren zu diagnostizieren und im Verlauf zu verfolgen. Die bioptische Diagnostik ist in diesem Zusammenhang nur in Ausnahmefällen erforderlich. Ultraschall und Magnetresonanztomografie zeigen den entzündlich bedingten Leberumbau auf. Nichtinvasive Messung der Steifigkeit/Elastizität der Leber erlauben Aussagen zum Ausmaß der Fibrose. Die bildgebenden Verfahren, ggf. ergänzt durch ERCP, erlauben es darüber hinaus, die Beschaffenheit der Gallengänge zu beurteilen. Sie sind damit auch wesentlicher Bestandteil zur Diagnose entzündlicher Cholangiopathien, insbesondere extrahepatischer Manifestationen der primären sklerosierenden Cholangitis (PSC) [8, 25, 37]. Kennzeichnende serologische Autoantikörper in Verbindung mit cholestatischem Leberenzymmuster begründen die Diagnose der primären biliären Cholangitis (PBC) ohne Notwendigkeit einer bioptischen Verifizierung [18, 37]. Tab. 1 Gegenüberstellung der 3 Haupttypen der Autoimmunhepatitis. (Nach Washington und Manns [43]) Typ Autoantikörper Alter zum Beginn (Jahre) Kommentar 1 ANA und/oder SMA Bimodal; mit Peaks 10-25 und 45-70 Nahezu 90 %, gutes Ansprechen auf Therapie 2 LKM-1, LKM-2, LC-1; ANA und SMA negativ <15 Etwa 10 %, Schwere Aktivität, nicht selten ungenügendes Ansprechen auf Therapie, häufig Relapse nach Therapie 3 SLA/LP Um 40 Selten, klinisch wie Typ 1

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“…According to major international guidelines, the diagnosis of PBC can be confidently made in patients with clinical, biochemical, and radiological evidence of intrahepatic cholestasis if they are positive for AMA [3, 4]. However, for patients negative for AMA, the diagnosis of PBC has to be based on typical pathological features of this disease [5, 6].…”

Section: Introductionmentioning

confidence: 99%

Meta-Analysis of Antinuclear Antibodies in the Diagnosis of Antimitochondrial Antibody-Negative Primary Biliary Cholangitis

Zhang

Liu

et al. 2019

Gastroenterology Research and Practice

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Objective. The diagnostic value of antinuclear antibodies (ANAs) including anti-gp210 and anti-sp100 for primary biliary cholangitis/cirrhosis (PBC) has been widely reported. However, their diagnostic performances for antimitochondrial antibody- (AMA-) negative PBC were less well elucidated. Therefore, the aim of the current meta-analysis was to evaluate the diagnostic accuracy of ANAs in patients with AMA-negative PBC. Materials and Methods. Literature on the diagnostic value of biomarkers for AMA-negative PBC was systematically searched in PubMed, MEDLINE, EMBASE, and the Cochrane Library. The qualities of the retrieved studies were assessed by the Quality Assessment of Diagnostic Accuracy Studies-version 2 (QUADAS-2) scale. Pooled sensitivity and specificity of the biomarkers were calculated with random-effects models. The areas under the summary receiver operating characteristic (AUSROC) curves were used to evaluate the overall diagnostic performance of ANAs. Results. A total of 11 studies (400 AMA-negative PBC patients and 6217 controls) were finally included in the meta-analysis. ANAs had an overall sensitivity of 27% (95% CI: 20%, 35%) and specificity of 98% (95% CI: 97%, 99%). The pooled sensitivities for anti-gp210 and anti-sp100 were 23% (95% CI: 13%, 37%) and 25% (95% CI: 13%, 43%), respectively, and their specificities were 99% (95% CI: 97%, 100%) and 97% (95% CI: 93%, 98%), respectively. Conclusions. ANAs exhibited high specificity but low sensitivity and therefore could be used as reliable biomarkers to reduce the necessity of liver histology.

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The diagnosis of antimitochondrial antibody-negative primary biliary cholangitis

Cited by 31 publications

References 42 publications

Emerging therapies for PBC

Emerging therapies for PBC

Autoimmune Lebererkrankungen

Meta-Analysis of Antinuclear Antibodies in the Diagnosis of Antimitochondrial Antibody-Negative Primary Biliary Cholangitis

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