Biochemical, histological parameters and response to UDCA treatment in primary biliary cirrhosis and primary biliary cirrhosis-autoimmune hepatitis overlap syndrome

Günşar, Fulya; Akarca, US; Ersöz, Galip; Karasu, Zeki; Yüce, Gül; Batur, Y.

doi:10.1016/s0168-8278(01)81677-9

Cited by 11 publications

(27 citation statements)

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“…Joshi et al [39] found that patients included in UDCA trials for PBC had similar improvement in biochemistries under UDCA monotherapy in patients with or without features of PBC-AIH overlap syndrome. Similarly, Gunsar et al [55] described that response to UDCA in patients with overlap syndrome was comparable with that obtained in PBC, and suggests that UDCA should be first-line therapy, while the combination of UDCA and immunosuppressants should be reserved to non-responders to UDCA. Once again, the heterogeneity in the criteria used to diagnose overlap syndromes in these studies might partially account for the variety of results obtained.…”

Section: Treatmentmentioning

confidence: 66%

“…Those include the response to corticosteroids as an additional feature of the Paris criteria [52] or the elimination of allocations of a negative score for the presence of AMA in revised scoring system [53]. Other authors have used yet different criteria for diagnosis such as Lohse et al [35], Suzuki et al [44] and Yamamoto et al [54], and Gunsar et al [55].…”

Section: Diagnostic Criteriamentioning

confidence: 99%

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Overlap Syndromes of Autoimmune Liver Disease

Silveira¹

2013

J Clin Cell Immunol

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Autoimmune hepatitis (AIH), primary biliary cirrhosis (PBC) and primary sclerosing cholangitis (PSC) are chronic liver diseases of presumed autoimmune origin. Conditions exhibiting features of two different autoimmune liver diseases are commonly designated overlap syndromes, although there is no current agreement on what constitutes an overlap syndrome or specific diagnostic criteria. Identification of patients with features of overlap syndrome is relevant for management as outcomes may differ from outcomes of patients with diagnosis of classic autoimmune liver diseases and treatment may need to be adjusted. Due to their rarity, no large therapeutic trials are available and treatment of overlap conditions is empirical and based upon extrapolation of data from the primary autoimmune liver diseases. AIH-PBC overlap is the most frequently described overlap syndrome and may be associated with a poor prognosis. This may represent an important and unrecognized cause of resistance to ursodeoxycholic acid (UDCA) in patients with PBC. AIH-PSC overlap is less commonly reported. Prognosis may be better than in patients with PSC alone, however worse than in patients with AIH alone. Further studies are needed for determining diagnosis, natural history and optimal therapeutic strategies of overlap syndromes of autoimmune liver diseases.

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Section: Treatmentmentioning

confidence: 66%

Section: Diagnostic Criteriamentioning

confidence: 99%

Overlap Syndromes of Autoimmune Liver Disease

Silveira¹

2013

J Clin Cell Immunol

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“…Günsar F, et al [14] reported that, lethargy was the most common symptom in these patients. Our study showed that xanthochromia, lethargy and anorexia were the predominant three chief complaints, that was, besides lethargy, xanthochromia was also a most common symptom in Chinese patients with overlap syndrome of AIH and PBC.…”

Section: Discussionmentioning

confidence: 99%

Low incidence of positive smooth muscle antibody and high incidence of isolated IgM elevation in Chinese patients with autoimmune hepatitis and primary biliary cirrhosis overlap syndrome: a retrospective study

Zhao¹,

Han²

2012

BMC Gastroenterol

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BackgroundUp to now, few data are available regarding the clinical characteristics of autoimmune hepatitis and primary biliary cirrhosis overlap syndrome. The study was to investigate and analyze the prevalent and clinical features of Chinese patients with this disease.MethodsClinical data on patients diagnosed as autoimmune hepatitis and primary biliary cirrhosis overlap syndrome in our hospital from January 2001 to December 2006 were collected and analyzed.ResultsOverlap syndrome of autoimmune hepatitis and primary biliary cirrhosis accounted for 10.33% of patients with autoimmune liver diseases during the past six years. For these patients with overlap syndrome, xanthochromia, lethargy and anorexia were the predominant complaints; a low incidence (14/146) of smooth muscle antibody positivity and a high incidence (37/89) of isolated IgM elevation were the main serological characteristics.ConclusionsOverlap syndrome of autoimmune hepatitis and primary biliary cirrhosis was not rare in Chinese patients with clinical manifests of autoimmune liver diseases. Overlap of the diseases should not be disregarded when isolated IgM elevation was exhibited, and smooth muscle antibody might have little diagnostic significance in the overlap syndrome. If it was difficult to make a definite diagnosis, liver biopsy was necessary.

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“…reported that response to UDCA therapy in patients with strictly defined PBC‐AIH overlap was similar to that of patients with PBC 6 . Some investigators have reported that immunosuppressive therapy is useful when UDCA therapy is ineffective 4,8 while others have suggested that combination therapy would be more effective at improving biological indicators than giving either UDCA or immunosuppressive therapy separately 8,17–19 . Until now, however, the effect of UDCA and immunosuppressive combination therapy on long term outcomes of PBC‐AIH overlap patients had not been evaluated.…”

Section: Discussionmentioning

confidence: 99%

“…It is currently acknowledged that patients with PBC‐AIH overlap should receive UDCA, with immunosuppressive therapy being used if the UDCA therapy is ineffective 4,16 . However, some researchers have suggested that UDCA and immunosuppressive combination therapy would be more effective in improving biological indicators for PBC‐AIH overlap patients than either UDCA or immunosuppressive therapy administered separately 8,17–19 . Additionally, a previous study indicated that patients with PBC and features of AIH overlap had a higher risk of symptomatic portal hypertension and worse outcomes compared to those with PBC alone 13 .…”

Section: Introductionmentioning

confidence: 99%

Overlap of primary biliary cirrhosis and autoimmune hepatitis: Characteristics, therapy, and long term outcomes

Yokokawa

Saito

Kanno

et al. 2010

J of Gastro and Hepatol

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Background: Coexistence of primary biliary cirrhosis (PBC) and autoimmune hepatitis (AIH) is referred to as PBC-AIH overlap. Pathogenesis of PBC-AIH is not well understood and its diagnosis is challenging. We previously reported the clinical characteristics of 10 patients diagnosed with PBC-AIH overlap. Aims: The aim of the study was extend the earlier series and evaluate the diagnostic criteria, biological characteristics, potential therapy, and long-term outcomes of patients with PBC-AIH overlap. Methods and Results:We retrospectively analyzed clinical, biochemical, and histological characteristics of 144 patients diagnosed with PBC and 73 diagnosed with AIH. We identified 16 cases of PBC-AIH overlap, according to criteria established by Chazouillères et al. and other studies. PBC preceded AIH in 6 patients and both diseases occurred simultaneously in the remaining 10 patients. PBC-AIH overlap has clinical, biochemical, and histological characteristics of both PBC and AIH. Thirteen patients treated with both ursodeoxycholic acid (UDCA) and immunosuppressive therapy responded well, with normal alanine aminotransferase (ALT) and alkaline phosphatase (ALP) levels. The remaining three patients treated with either prednisolone (PSL) or UDCA alone developed cirrhosis, varices, ascites, encephalopathy, or died of liver-related causes at the 5, 12, and 14-year follow up. Conclusions: PBC-AIH overlap is not a rare entity; it was observed in 11% of PBC patients in this study. Further studies will be required to investigate whether PBC-AIH overlap is distinct from the two individual diseases in terms of long-term outcomes and therapeutic implications.

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Biochemical, histological parameters and response to UDCA treatment in primary biliary cirrhosis and primary biliary cirrhosis-autoimmune hepatitis overlap syndrome

Cited by 11 publications

References 0 publications

Overlap Syndromes of Autoimmune Liver Disease

Overlap Syndromes of Autoimmune Liver Disease

Low incidence of positive smooth muscle antibody and high incidence of isolated IgM elevation in Chinese patients with autoimmune hepatitis and primary biliary cirrhosis overlap syndrome: a retrospective study

Overlap of primary biliary cirrhosis and autoimmune hepatitis: Characteristics, therapy, and long term outcomes

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