Antinuclear antibodies directed to a 200-kilodalton polypeptide of the nuclear envelope in primary biliary cirrhosis

Lassoued, Kaı̈ss; Brenard, R.; Degos, Françoise; Courvalin, Jean-Claude; André, Chantal; Danon, F; Brouet, Jean‐Claude; Zine-El-Abidine, Yousser; Degott, Claude; Zafrani, Serge; Dhumeaux, Daniel; Benhamou, Jean‐Pierre

doi:10.1016/0016-5085(90)91246-3

Cited by 84 publications

(41 citation statements)

References 19 publications

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“…9 Several studies have consistently demonstrated that AMA titers or patterns do not correlate with PBC stage or prognosis, [9][10][11] whereas it is still debated if antinuclear antibodies (ANAs) directed against antigens of the nuclear pore complex (anti-NPC) or "nuclear dot" (anti-ND) associate with features of more severe disease. In fact, older studies failed to demonstrate such association 12,13 ; however, we note that these studies included anti-NPC specificities only in a subgroup of patients. On the other hand, more recent studies suggested an association between these serum reactivities and features of a more severe and active disease [14][15][16] , in some cases depending also on immunoglobulin isotypes.…”

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confidence: 66%

Correlation of initial autoantibody profile and clinical outcome in primary biliary cirrhosis

et al. 2006

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Although there have been significant advances in understanding the clinical and biochemical features of primary biliary cirrhosis (PBC), there is still a paucity of data on the usefulness of biomarkers as prognostic indicators. This is particularly important at the time of initial diagnosis. Indeed, the widespread use of antimitochondrial antibody testing has led to an earlier diagnosis of asymptomatic PBC and it is difficult to predict which patients will experience a benign versus a rapidly progressive course. To address this issue, we examined a unique population of 127 newly diagnosed patients with PBC during a 15-year period of observation that began in January 1990. Sera from these patients were analyzed for antimitochondrial, antinuclear, and anti-smooth muscle antibodies, and immunoblotting was performed for nuclear pore complex (NPC). The patients were then followed up longitudinally using biochemical liver function tests. No patient was under any medical therapy for PBC at the time of the initial sera collection. Data were analyzed based not only on the clinical features, but also the Mayo score and specific outcome measures, including time to death, need for liver transplantation, and complication free survival. Among patients with early disease, bilirubin increased to >2 mg/dL in the anti-NPC(؉) patients (26% vs. 5%, P ‫؍‬ .019). Anti-NPC antibodies remained stable or slightly increased over the period of observation. In conclusion, anti-NPC identifies patients likely to experience an unfavorable clinical course and more rapid disease progression. (HEPATOLOGY 2006;43:1135-1144

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confidence: 66%

Correlation of initial autoantibody profile and clinical outcome in primary biliary cirrhosis

et al. 2006

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“…However, compared with the high frequency of AMA in sera and bile of patients with PBC, the incidence of antigp210 (nuclear envelope protein) autoantibodies in PBC sera tested was low and is consistent with the reported incidence in the United States, 29 but less than the incidence reported in two European studies. 30,31 IgA autoantibodies against the predominant autoepitope of gp210 were not detected in bile. Elevation of serum polymeric IgA concentration is common in PBC.…”

Section: Detection Of Mitochondrial Autoantigens In Sera and Bilementioning

confidence: 95%

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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“…In human diseases polyclonal autoantibodies to nuclear envelope polypeptides are mainly directed to a 200-kD gly coprotein (NEp200) of the membrane pore complex or to lamins A, B or C [3][4][5], Autoantibodies to Ncp200 appear to be a specific marker of primary biliary cirrhosis [4,6], whereas autoantibodies to lamins can be detected in vari ous autoimmune disorders, usually associated with periph eral blood cytopenias, vasculitis, acute or chronic hepatitis such as primary biliary cirrhosis or autoimmune hepatitis [3,7,8]. We report here a patient with anti-lamin-B autoantibodies associated with idiopathic cold urticaria.…”

Section: Discussionmentioning

confidence: 99%

Anti-Lamin-B Autoantibodies in a Patient with Cold Urticaria

et al. 1992

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Anti-lamin-B autoantibodies at a significant level had been found on two occasions in the serum of a 56-year-old woman who was suffering from an apparently idiopathic chronic cold urticaria. Anti-lamin autoantibodies can be detected in various autoimmune disorders including hepatitis, vasculitis and peripheral blood cytopenia. In our patient, there was no other clinical or biological abnormality. A chance association cannot be ruled out.

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Antinuclear antibodies directed to a 200-kilodalton polypeptide of the nuclear envelope in primary biliary cirrhosis

Cited by 84 publications

References 19 publications

Correlation of initial autoantibody profile and clinical outcome in primary biliary cirrhosis

Correlation of initial autoantibody profile and clinical outcome in primary biliary cirrhosis

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

Anti-Lamin-B Autoantibodies in a Patient with Cold Urticaria

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