The Serological Profile of the Autoimmune Hepatitis/Primary Biliary Cirrhosis Overlap Syndrome

Muratori, Paolo; Granito, Alessandro; Pappas, Georgios; Pendino, Gaspare Maria; Quarneti, Chiara; Cicola, Ronny; Menichella, R.; Ferri, Silvia; Cassani, F.; Bianchi, Francesco; Lenzi, Marco; Muratori, Luigi

doi:10.1038/ajg.2009.126

Cited by 112 publications

(64 citation statements)

References 35 publications

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“…In a recent study, anti-dsDNA antibodies were significantly more frequent in 15 PBC-AIH overlap cases (60%) than in 120 PBC (4%)-and 120 AIH (26%) patients (<0.0001 and 0.01, respectively) [109]. The concomitant presence of AMA and anti-dsDNA was highly specific (98%) for the overlap condition, being observed in 47% of overlaps as compared to only 2% of controls.…”

Section: International Study Of Patients With Autoimmune Liver Disordersmentioning

confidence: 94%

Overlap syndromes: The International Autoimmune Hepatitis Group (IAIHG) position statement on a controversial issue

Boberg

Chapman

Hirschfield

et al. 2011

Journal of Hepatology

468

411

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Some patients present with overlapping features between disorders within the spectrum of autoimmune liver diseases (i.e. autoimmune hepatitis (AIH), primary biliary cirrhosis (PBC), and primary sclerosing cholangitis (PSC)) and are commonly classified as having an "overlap syndrome". Standardized definitions of "overlap syndromes" are lacking. The aim of this report by the International Autoimmune Hepatitis Group (IAIHG) is to evaluate if there are important reasons to classify conditions with overlapping features between autoimmune liver diseases as separate diagnostic entities. Definition of diagnostic criteria for overlap conditions can only be arbitrary. The IAIHG scoring system for diagnosis of AIH has been widely used to diagnose "overlap syndromes", but was not intended for such use and has not proven to be an efficient tool for this purpose. Some patients with overlapping features between a cholestatic and hepatitic disorder appear to benefit from treatment with a combination of ursodeoxycholic acid and immunosuppressants, but this strategy is not evidence-based, and it seems unjustified to define new diagnostic groups in this regard. The IAIHG suggests that patients with autoimmune liver disease should be categorized according to the predominating feature(s) as AIH, PBC, and PSC/small duct PSC, respectively, and that those with overlapping features are not considered as being distinct diagnostic entities. The IAIHG scoring system should not be used to establish subgroups of patients. Patients with PBC and PSC with features of AIH should be considered for immunosuppressive treatment. Due to the low prevalence of such "overlap syndromes", prospective interventional therapeutic trials cannot be expected in the foreseeable future.

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Section: International Study Of Patients With Autoimmune Liver Disordersmentioning

confidence: 94%

Overlap syndromes: The International Autoimmune Hepatitis Group (IAIHG) position statement on a controversial issue

Boberg

Chapman

Hirschfield

et al. 2011

Journal of Hepatology

468

411

View full text Add to dashboard Cite

show abstract

“…In addition, Zhao et al reported that 41.5% patients with PBC -AIH overlap showed isolated IgM elevation 9) . These findings were not reported from Western countries 2,3,8) , but we believe in the importance in studying elevation of IgM and its relationship with the onset of PBC -AIH overlap.…”

Section: Discussionmentioning

confidence: 60%

“…Elevated IgM is thus a common finding, as in PBC 1,2,3,5) . However, it is unusual to see an increase in IgM as high as the level of 4,250 mg/dl seen in this case or higher than the IgG level.…”

Section: Discussionmentioning

confidence: 99%

A CASE OF OVERLAP SYNDROME OF PRIMARY BILIARY CIRRHOSIS AND AUTOIMMUNE HEPATITIS WITH MARKED ELEVATION OF IgM

Katsushima

Takahashi

Suzuki

et al. 2013

FJMS

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: A 60 -year -old woman was admitted to our hospital because of anorexia and vomiting. Hematology revealed elevated levels of hepatobiliary enzymes and positive results for antinuclear antibody (ANA) and antimitochondrial (AMA) M2 antibody. Immunoglobulin (Ig) G and IgM levels were extremely elevated, 3,379 mg/dl and 4,250 mg/dl, respectively. A diagnosis of primary biliary cirrhosis and autoimmune hepatitis (PBC -AIH) overlap syndrome was made. However, IgM levels as high as the level of 4,250 mg/dl in this case has not been reported previously. This case may be of value in studying elevated IgM levels with PBC -AIH overlap syndrome and relationships to the mechanism of onset.

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“…Furthermore, antidsDNA antibodies, which are known to be strongly associated with SLE, were detected in 60% [50] or 56% [51] of patients with AIH-PBC overlap syndrome.…”

Section: Sle-pbc Overlap Syndromementioning

confidence: 99%

Challenge of liver disease in systemic lupus erythematosus: Clues for diagnosis and hints for pathogenesis

Bessone

Poles

Roma

2014

WJH

106

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Systemic lupus erythematosus (SLE) encompass a broad spectrum of liver diseases. We propose here to classify them as follows: (1) immunological comorbilities (overlap syndromes); (2) non-immunological comorbilities associated to SLE; and (3) a putative liver damage induced by SLE itself, referred to as "lupus hepatitis". In the first group, liver injury can be ascribed to overlapping hepatopathies triggered by autoimmune mechanisms other than SLE occurring with higher incidence in the context of lupus (e.g. , autoimmune hepatitis, primary biliary cirrhosis). The second group includes non-autoimmune liver diseases, such as esteatosis, hepatitis C, hypercoagulation state-related liver lesions, hyperplasic parenchymal and vascular lesions, porphyria cutanea tarda, and drug-induced hepatotoxicity. Finally, the data in the literature to support the existence of a hepatic disease produced by SLE itself, or the occurrence of a SLE-associated prone condition that increases susceptibility to acquire other liver diseases, is critically discussed. The pathological mechanisms underlying each of these liver disorders are also reviewed. Despite the high heterogeneity in the literature regarding the prevalence of SLE-associated liver diseases and, in most cases, lack of histopathological evidence or clinical studies large enough to support their existence, it is becoming increasingly apparent that liver is an important target of SLE. Consequently, biochemical liver tests should be routinely carried out in SLE patients to discard liver disorders, particularly in those patients chronically exposed to potentially hepatotoxic drugs. Diagnosing liver disease in SLE patients is always challenging, and the systematization of the current information carried out in this review is expected to be of help both to attain a better understanding of pathogenesis and to build an appropriate work-up for diagnosis.

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The Serological Profile of the Autoimmune Hepatitis/Primary Biliary Cirrhosis Overlap Syndrome

Abstract: Concomitant AMA/anti-dsDNA seropositivity can be considered the serological profile of AIH/PBC OS. The combination of UDCA and steroids is effective in achieving persistent biochemical amelioration in most AIH/PBC OS patients.

Cited by 112 publications

References 35 publications

Overlap syndromes: The International Autoimmune Hepatitis Group (IAIHG) position statement on a controversial issue

Overlap syndromes: The International Autoimmune Hepatitis Group (IAIHG) position statement on a controversial issue

A CASE OF OVERLAP SYNDROME OF PRIMARY BILIARY CIRRHOSIS AND AUTOIMMUNE HEPATITIS WITH MARKED ELEVATION OF IgM

Challenge of liver disease in systemic lupus erythematosus: Clues for diagnosis and hints for pathogenesis

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