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    Abstract: Introduction: Anti-glomerular basement membrane (GBM) antibodies are pathogenic antibodies first detected in renal-limited anti-GBM disease and in Goodpasture disease, the latter characterized by rapidly progressive crescentic glomerulonephritis combined with intra-alveolar hemorrhage. Studies have suggested that anti-GBM antibody positivity may be of interest in lupus nephritis (LN). Moreover, severe anti-GBM vasculitis cases in patients with systemic lupus erythematosus (SLE) have been described in the literature, but few studies have assessed the incidence of anti-GBM antibodies in SLE patients. Objective: The main study objective was to determine if positive anti-GBM antibodies were present in the serum of SLE patients with or without proliferative renal damage and compared to a healthy control group. Methodology: This retrospective study was performed on SLE patients' sera from a Franco-German European biobank, developed between 2011 and 2014, from 17 hospital centers in the Haut-Rhin region. Patients were selected according to their renal involvement, and matched by age and gender. The serum from healthy voluntary blood donors was also tested. Anti-GBM were screened by fluorescence enzyme immunoassay (FEIA), and then by indirect immunofluorescence (IIF) in case of low reactivity detection (titer >6 U/ml). Results: The cohort was composed of 100 SLE patients with proliferative LN (27% with class III, 67% with class IV, and 6% with class V), compared to 100 SLE patients without LN and 100 controls. Patients were mostly Caucasian and met the ACR 1997 criteria and/or the SLICC 2012 criteria. Among the 300 tested sera, no significant levels of anti-GBM antibodies were detected (>10 U/ml) by the automated technique, three sera were found "ambivalent" (>7 U/ml): one in the SLE with LN group and two in the SLE without LN group. Subsequent IIF assays did not detect anti-GBM antibodies. Conclusion: Anti-GBM antibodies were not detected in the serum of Caucasian patients with SLE, even in case of renal involvement, a situation favoring the antigenic exposure of glomerular basement membranes. Our results reaffirm the central role of anti-GBM antibodies as a specific diagnostic biomarker for Goodpasture vasculitis and therefore confirm that anti-GBM antibody must not be carried out in patients with SLE (with or without LN) in the absence of disease-suggestive symptoms

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    LA CHORIORETINOPATHIE DE BIRDSHOT (CRBS) EST UNE UVEITE POSTERIEURE RARE N'AFFECTANT QUE LES SUJETS CAUCASIENS. ELLE RESSEMBLE A L'UVEITE AUTOIMMUNE EXPERIMENTALE (UAE). LA PRESENCE QUASI PERMANENTE DE L'ANTIGENE D'HISTOCOMPATIBILITE DE CLASSE I HLA-A29 CHEZ CES PATIENTS EN FAIT UN MODELE D'ETUDE DES RELATIONS ENTRE HLA ET MALADIE. IL EXISTE DEUX SOUS-TYPES HLA-A29 INEGALEMENT REPARTIS AU SEIN DES CAUCASIENS: CINQ SONT A*2902 POUR UN A*2901. L'ETUDE PAR ISOELECTROFOCALISATION D'IMMUNOPRECIPITES D'EXTRAITS MEMBRANAIRES APRES MARQUAGE BIOSYNTHETIQUE PAR LA METHIONINE #3#5S DE LIGNEES LYMPHOBLASTOIDES DE 33 MALADES MONTRE QU'ILS POSSEDENT TOUS L'ALLOTYPE HLA-A*2902. LA SEQUENCE HLA-A*2902 MONTRE QUE L'ADN COMPLEMENTAIRE DES MALADES EST IDENTIQUE A CELUI DES SUJETS SAINS. LA SEQUENCE PROTEIQUE QUI EN DECOULE NE MONTRE QU'UNE SUBSTITUTION DANS LE DOMAINE EXTRACELLULAIRE DES DEUX SOUS-TYPES EN POSITION 102: A29.2 PARTAGE LE CONCENSUS HLA DE CLASSE I (D#1#0#2), A29.1 EST UNIQUE (H#1#0#2). LE SOUS-TYPE ANCESTRAL EST PROBABLEMENT A29.2 ; A29.1 SERAIT LE PRODUIT D'UNE MUTATION RECENTE. DES CELLULES LJ26 DE SOURIS, FIBROBLASTES EXPRIMANT LA BETA-2 MICROGLOBULINE HUMAINE A LEUR SURFACE, ONT ETE TRANSFECTEES PAR LES CLONES D'ADN COMPLEMENTAIRE DES DEUX SOUS-TYPES. AUCUN SOUS-TYPE NE PRESENTE DE DEFAUT DE LIAISON AUX CHAINES ALPHA DE CD8 DANS UN TEST IN VITRO DE LIAISON INTER-CELLULAIRE A DES CELLULES CHO EXPRIMANT CD8. CES CELLULES LJ26 HLA-A*2902 ONT PERMIS DE DEFINIR LES MOTIFS D'ANCRAGES PREFERENTIELS DES PEPTIDES ELUES (RESPECTIVEMENT E, F, Y EN POSITIONS 2, 3, 9) ET DE TESTER LA LIAISON DE PEPTIDES REPUTES UVEITOGENES. DES SOURIS TRANSGENIQUES D'ADN COMPLEMENTAIRE DES DEUX SOUS-TYPES TRANSCRIVENT LE TRANSGENE EN ARN, MAIS N'EXPRIMENT PAS LA PROTEINE HLA-A29 QUE LE GENE SOIT SOUS LE CONTROLE DU PROMOTEUR DE LA RHODOPSINE BOVINE OU D'H-2K#B. CE BLOQUAGE D'EXPRESSION SERA ETUDIE PAR COMPARAISON DES TRANSGENES D'ADN GENOMIQUE EN COURS DE DEVELOPPEMENT.

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