title: Cogan's syndrome: anti-Hsp70 antibodies are a serological marker in the typical form.
abstract: BACKGROUND
  Cogan's syndrome (CS) is a rare autoimmune vasculitis characterized by ocular inflammation and sensorineural hearing loss. CS is divided into a "typical" form with non-syphilitic interstitial keratitis and audiovestibular symptoms, and an "atypical" form with ocular involvement affecting structures other than the cornea. Anti-Hsp70 antibodies were found at variable levels in patients presenting with various forms of autoimmune sensorineural hearing loss (ASNHL).
  
  
  OBJECTIVE
  To assess the correlation between anti-Hsp70 antibodies and specific ASNHL subgroups.
  
  
  METHODS
  We divided 112 subjects into four groups: 14 subjects with typical CS, 24 with atypical CS, 55 with ASNHL, and 19 control subjects (healthy subjects and patients with systemic autoimmune diseases but no sensorineural hearing or audiovestibular alterations). Patients were tested for serological autoimmunity markers including anti-Hsp70.
  
  
  RESULTS
  Positivity of the anti-Hsp70 antibody test was highest in the typical CS group (92.9%) and lowest in the control group (5.2%). The test was positive in 52.7% of patients in the ASNHL group and 16.6% in the atypical CS group. The paired comparison analysis between groups showed that sensitivity of anti-Hsp70 in the typical CS group was significantly higher, as compared to the other three study groups.
  
  
  CONCLUSIONS
  Anti-Hsp70 antibodies can be considered a serological marker of "typical" CS. "Atypical" CS is conceivably a sort of "melting pot" of different forms of autoimmune diseases still characterized by ocular inflammation and sensorineural hearing loss but whose antigenic characteristics need to be further defined.
authors: Chiara
authors: C
authors: Bonaguri
authors: Jelka
authors: J
authors: Orsoni
authors: Annalisa
authors: A
authors: Russo
authors: Pierangela
authors: P
authors: Rubino
authors: Salvatore
authors: S
authors: Bacciu
authors: Giuseppe
authors: G
authors: Lippi
authors: Alessandra
authors: A
authors: Melegari
authors: Laura
authors: L
authors: Zavota
authors: Stella
authors: S
authors: Ghirardini
authors: Paolo
authors: P
authors: Mora
mesh_terms: D000328:Adult; D000368:Aged; D001323:Autoantibodies / Q000097:blood*; D015551:Autoimmunity / Q000276:immunology; D015415:Biomarkers / Q000097:blood; D016009:Chi-Square Distribution; D002648:Child; D055952:Cogan Syndrome / Q000145:classification / Q000276:immunology / Q000503:physiopathology; D005260:Female; D018840:HSP70 Heat-Shock Proteins / Q000276:immunology*; D006801:Humans; D008297:Male; D012680:Sensitivity and Specificity
chemical_list: D001323:Autoantibodies; D015415:Biomarkers; D018840:HSP70 Heat-Shock Proteins