title: Autoantibodies against lamin C, NA14 and CK15 in primary vasculitides or autoimmune diseases with secondary vasculitis.
abstract: OBJECTIVE
  Autoantibodies may play a role in the pathogenesis of primary vasculitides (PVs) like giant cell arteritis (GCA). We recently identified 3 cytoskeletal proteins (lamin C [laC], nuclear autoantigen of 14kD [NA14] and cytokeratin 15 [CK15]) as autoantigens in GCA and postulated a frequent autoantibody response against these antigens in PVs.
  
  
  METHODS
  To test this hypothesis we studied a cohort of patients with PVs (n=61) and healthy individuals (n=27) for the presence of these autoantibodies using a recombinant cDNA expression library. To define their specifity for PV, we also examined patients with other autoimmune diseases such as rheumatoid arthritis (RA) and connective tissue diseases (CTD).
  
  
  RESULTS
  We found no statistically significant differences in autoantibody responses between patients with PV and healthy controls, although there was a trend for an association between PVs and the occurrence of antibodies against laC and CK15. However, in patients with RA (n=33) or Sjögren's syndrome (SS, n=11) with vasculitides we observed more frequently autoantibodies against NA14, laC and CK15 compared to healthy controls. In patients with systemic lupus erythematosus (SLE, n=23) autoantibodies against laC were more frequent. The presence of autoantibodies in RA, SS and SLE was associated with systemic secondary vasculitis (SSV). In RA, laC- and NA14-seropositive patients were in a more advanced disease stage than seronegative patients with more frequent extraarticular manifestations (p=0.004). In SLE laC-autoantibody-positive patients had a higher SLE activity index (p<0.001).
  
  
  CONCLUSIONS
  Serum autoantibodies against laC and NA14 are frequent in patients with RA and CTD and are associated with extensive disease and SSV. The potential pathogenic and prognostic role of these antibodies should be further investigated.
authors.affiliation: Department of Internal Medicine I, José-Carreras Centrum for Immuno- and Gene Therapy, University of Saarland Medical School, Homburg/Saar, Germany. boris.kubuschok@uks.eu.
authors.forename: Boris
authors.initials: B
authors.lastname: Kubuschok
authors.affiliation: Department of Internal Medicine I, José-Carreras Centrum for Immuno- and Gene Therapy, University of Saarland Medical School, Homburg/Saar, Germany.
authors.forename: Klaus-Dieter
authors.initials: KD
authors.lastname: Preuss
authors.affiliation: Department of Internal Medicine I, José-Carreras Centrum for Immuno- and Gene Therapy, University of Saarland Medical School, Homburg/Saar, Germany.
authors.forename: Kerstin
authors.initials: K
authors.lastname: Baier-Thoenes
authors.affiliation: Department of Internal Medicine I, José-Carreras Centrum for Immuno- and Gene Therapy, University of Saarland Medical School, Homburg/Saar, Germany.
authors.forename: Evi
authors.initials: E
authors.lastname: Regitz
authors.affiliation: Department of Internal Medicine I, José-Carreras Centrum for Immuno- and Gene Therapy, University of Saarland Medical School, Homburg/Saar, Germany.
authors.forename: Lorenz
authors.initials: L
authors.lastname: Thurner
authors.affiliation: Department of Internal Medicine I, José-Carreras Centrum for Immuno- and Gene Therapy, University of Saarland Medical School, Homburg/Saar, Germany.
authors.forename: Gunter
authors.initials: G
authors.lastname: Assmann
authors.affiliation: Department of Internal Medicine I, José-Carreras Centrum for Immuno- and Gene Therapy, University of Saarland Medical School, Homburg/Saar, Germany.
authors.forename: Michael
authors.initials: M
authors.lastname: Pfreundschuh
mesh_terms: D000328:Adult; D000368:Aged; D001323:Autoantibodies / Q000097:blood*; D001324:Autoantigens / Q000276:immunology*; D001327:Autoimmune Diseases / Q000276:immunology*; D005260:Female; D006801:Humans; D007074:Immunoglobulin G / Q000097:blood; D053569:Keratin-15 / Q000276:immunology*; D034904:Lamin Type A / Q000276:immunology*; D008297:Male; D008875:Middle Aged; D009687:Nuclear Proteins / Q000276:immunology*; D014657:Vasculitis / Q000276:immunology*
chemical_list: D001323:Autoantibodies; D001324:Autoantigens; D007074:Immunoglobulin G; D053569:Keratin-15; D034904:Lamin Type A; C110204:NA14 nuclear autoantigen; D009687:Nuclear Proteins; C440308:lamin C