Primary Biliary Cirrhosis and Cœliac Disease

Logan, Richard F.; Ferguson, Anne; Finlayson, N. D. C.; Weir, D. G.

doi:10.1016/s0140-6736(78)90480-4

Cited by 131 publications

(52 citation statements)

References 9 publications

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“…The association of CD and primary biliary cirrhosis (PBC) was reported by Logan et al in 1978. 29 Subsequently, the association between CD and PBC has been extensively investigated by screening patients with PBC for CD. The reported prevalence of CD in PBC varies widely (0%-11%; Table 3).…”

Section: Primary Biliary Cirrhosismentioning

confidence: 99%

The liver in celiac disease

2007

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Celiac disease is a common (1% prevalence) chronic immune-mediated disorder of the small intestine induced by dietary wheat, barley, and rye. Several hepatic disorders have been described in association with celiac disease. Isolated hypertransaminasemia with nonspecific histologic changes in a liver biopsy is the commonest hepatic presentation of celiac disease. A gluten-free diet normalizes liver enzymes and histologic changes in most patients. Moreover, celiac disease can coexist with autoimmune liver disorders such as autoimmune hepatitis, primary biliary cirrhosis, and primary sclerosing cholangitis. Celiac disease has increasingly been reported with a variety of other liver diseases. Thus, the hepatologist needs to consider celiac disease in the differential of abnormal liver blood tests and to be aware of the clinical implications of this frequent disease in patients with liver disorders. The possible mechanisms of liver injury and those common factors that explain the association of celiac disease with liver disorders are discussed. The aims of this article are (1) to review the spectrum and pathogenesis of liver injury related to celiac disease and (2) to provide direction to those caring for patients with chronic liver diseases regarding the detection and effective treatment of celiac disease. (HEPATOLOGY 2007;46:1650-1658

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Section: Primary Biliary Cirrhosismentioning

confidence: 99%

The liver in celiac disease

2007

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“…This hypothesis is also consistent with reports of PBC in patients with selective and severe serum IgA deficiency, although serum IgA deficiency may not correlate with IgA deficiency in bile. 36,37 We must note, however, that the patients studied herein were examined late in the disease process, when few viable biliary epithelial cells remain. At this point, there may be little IgA transported into bile because of this reduction in both the number and functionality of toantigens of PBC, in the bile of patients with PBC.…”

Section: Detection Of Mitochondrial Autoantigens In Sera and Bilementioning

confidence: 99%

Comparative studies of antimitochondrial autoantibodies in sera and bile in primary biliary cirrhosis

et al. 1997

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9%), to the branched-chain 2-oxo-acid dehydrogenase complex E2 (BCOADC-E2) in 6 of 19 patientsOrgan-specific autoimmune diseases are characterized by (31.6%), and to the 2-oxoglutarate dehydrogenase com-the production of autoantibodies and the destruction of speplex E2 (OGDC-E2) in 1 of 19 patients (5.3%). In a compar-cific tissues. Primary biliary cirrhosis (PBC) has been considative study of sera from the same patients, anti-PDC-ered a model autoimmune disease and a paradigm for dis-E2 antibodies were found in 19 of 19 patients (100%), eases such as Hashimoto's thyroiditis 1 and type I diabetes anti-BCOADC in 9 of 19 patients (47.3%), and anti-mellitus. 2 PBC is characterized by destruction of intrahepatic OGDC-E2 in 4 of 19 patients (21.1%) patients. AMA in bile ducts and production of high-titer autoantibodies against bile were always found together with antibodies of cor-mitochondrial autoantigens. 3 Much work has been directed responding specificities in the serum from the same pa-at the detection and characterization of antimitochondrial tient. Immunoglobulin (Ig)A AMA were found in the bile autoantibodies (AMA). Earlier studies from both our laboraof 9 of 19 patients (47.7%) with PBC; they were specifi-tory and others have shown that AMA react with a series of cally directed against PDC-E2 in 8 of 19 patients (42.1%) highly conserved intramitochondrial proteins, in particular and to BCOADC in 2 of 19 patients (10.5%). Epitope map-the 2-oxo acid dehydrogenase complexes, including the dihyping of IgA anti-PDC-E2 antibodies indicated that, like drolipoylacetyltransferase component of the pyruvate dehyserum autoantibodies, the immunodominant epitope is drogenase complex E2 (PDC-E2), the branched-chain 2-oxo directed against the inner lipoyl domain of PDC-E2. The acid dehydrogenase complex E2 (BCOADC-E2), and the 2-prevalence and antigen reactivity of IgA AMA in sera oxoglutarate dehydrogenase complex E2 (OGDC-E2). [4][5][6][7][8][9][10][11] The correlated completely with IgA AMA in bile. Autoanti-major autoantibody reactivity is directed against the 74-kd bodies against nuclear envelope pore proteins (gp210) protein, PDC-E2. were found in 1 of 8 (12.5%) sera of patients with PBC, Although the pathogenesis of PBC remains unknown, utilibut not in bile. Furthermore, and of particular interest, zation of immunohistochemical techniques, monoclonal, combinatorial derived, and experimentally induced rabbit antibodies specific for PDC-E2 has revealed the selective expression of a cross-reactive molecule at the apex of biliary Abbreviations: PBC, primary biliary cirrhosis; AMA, antimitochondrial autoantibodies; PDC, pyruvate dehydrogenase complex; BCOADC, branched-chain 2-oxo-acid dehydrogeepithelial cells of patients with PBC, but not patients with nase complex; OGDC, 2-oxoglutarate dehydrogenase complex; Ig, immunoglobulin; PBS, primary sclerosing cholangitis or autoimmune hepatitis. 12-14 phosphate-buffered saline; SDS, sodium dodecyl sulfate; BHM, bovine heart mitochondrial Furthermore, deposition of immunoglobul...

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“…Eles enfatizaram a importância de se reconhecer que essas duas afecções podem ocorrer simultaneamente e que a perda ponderal, nesses pacientes, pode não ser por esteatorréia secundária à redução da secreção de ácidos biliares, e sim pela enteropatia glúten-sensível. 19 Existe uma relação entre Doença Celíaca e os genes de histocompatibilidade, especialmente os fenótipos HLA B8, HLA DR3, HLA DR7 e DQW2. Numerosas doenças têm sido descritas em associação com DC, e a maioria delas é de origem auto-imune, associadas com alguns haplótipos dessa enteropatia.…”

Section: Hipertransaminasemiaunclassified

Alterações hepáticas na doença celíaca

Franca¹,

Diniz-Santos²,

May³

et al. 2006

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ResumoO envolvimento do fígado tem sido freqüentemente descrito em pacientes celíacos. Condições como hipertransaminasemia, que retorna a valores normais após a dieta isenta de glúten, doenças hepáticas de origem auto-imune e outras doenças crônicas do fígado, sobretudo a hepatite crônica pelo vírus C, estão associadas com a doença celíaca. O objetivo deste artigo é discutir as relações recentemente evidenciadas na literatura entre essa enteropatia glúten-sensível e os tipos de alterações hepáticas. Palavras INTRODUÇÃOUm grande número de pacientes adultos e crianças com Doença Celíaca (DC) apresenta alterações hepáticas. O envolvimento do fígado em pacientes celíacos pode ser demonstrado de várias formas; pode haver hipertransaminasemia, que retorna, a valores normais após a dieta isenta de glúten, alterações que configuram uma doença hepática de origem auto-imune, bem como associação com outras doenças crônicas do fígado. Este estudo faz uma revisão dos aspectos descritos até o momento e da patogênese nesses tipos de envolvimento do fígado que podem ocorrer na DC.Em 1990, a partir da revisão dos critéri-os diagnósticos atualizados para Doença celíaca, estabelecidos pela ESPGAN (Sociedade Européia de Gastroenterologia Pediátrica e Nutrição), adotou-se uma abordagem prática e sistematizada para o diagnóstico da doença celíaca. São considerados indispensáveis para o diagnós-tico da DC os achados de alterações caracterís-ticas na mucosa intestinal em, no mínimo, um espécime de biópsia, achados esses que devem estar associados à presença de marcadores sorológicos positivos, havendo, na evolução do paciente, remissão clínica dos sintomas e negativação da sorologia, quando instituída a dieta isenta de glúten. 1 A Doença Celíaca apresenta um amplo espectro de manifestações clínicas, o que torna o seu diagnóstico um desafio em muitas situações. As formas clássicas, consideradas estágios

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Primary Biliary Cirrhosis and Cœliac Disease

Cited by 131 publications

References 9 publications

The liver in celiac disease

The liver in celiac disease

Comparative studies of antimitochondrial autoantibodies in sera and bile in primary biliary cirrhosis

Alterações hepáticas na doença celíaca

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