title: Chronic periaortitis and HLA-DRB1*03: another clue to an autoimmune origin.
abstract: OBJECTIVE
  Patients with chronic periaortitis (CP) often show clinical and laboratory findings of a systemic autoimmune disorder. The aim of the present study was to investigate the role of the HLA system in CP.
  
  
  METHODS
  Low-resolution genotyping for HLA-A, HLA-B, and HLA-DRB1 loci and genotyping of TNFA(-238)A/G and TNFA(-308)A/G single nucleotide polymorphisms were performed in 35 consecutive patients with CP and 350 healthy controls.
  
  
  RESULTS
  The HLA-DRB1*03 allele frequency was strikingly higher in patients with CP than in controls (24.28% versus 9.14%; chi(2) = 15.50, P = 0.000084, corrected P [P(corr)] = 0.0012, odds ratio [OR] 3.187, 95% confidence interval [95% CI] 1.74-5.83); the HLA-B*08 allele frequency was also higher in patients than in controls (17.14% versus 6.28%; chi(2)=11.12, P = 0.0008, P(corr) = 0.0269, OR 3.085, 95% CI 1.54-6.16). The A*01 allele frequency was significantly different (P = 0.0463), but the statistical significance was lost after correction for multiple testing (P(corr) = 0.5088). TNFA(-238)A allele and TNFA(-308)A allele frequencies were not significantly different (P = 0.512 and P = 0.445, respectively). Comparison of the main clinical and laboratory findings suggestive of a systemic autoimmune disease (e.g., acute-phase reactants, constitutional symptoms, other autoimmune diseases associated with CP) between the HLA-DRB1*03-positive and the HLA-DRB1*03-negative patients showed that the former group had significantly higher levels of C-reactive protein (P = 0.045) at disease onset, although this difference was not statistically significant after correction for multiple tests (P(corr) = 0.369).
  
  
  CONCLUSIONS
  The HLA system plays a role in susceptibility to CP. The strong association between CP and HLA-DRB1*03, an allele linked to a wide range of autoimmune conditions, further supports the view that CP may represent a clinical manifestation of an autoimmune disease.
authors.affiliation: Laboratory of Molecular Genetiucs, Dept. of Clinical Medicine, Nephrology and Health Science, University of Parma, Via Gramsci 14, 43100 Parma, Italy.
authors.forename: Davide
authors.initials: D
authors.lastname: Martorana
authors.forename: Augusto
authors.initials: A
authors.lastname: Vaglio
authors.forename: Paolo
authors.initials: P
authors.lastname: Greco
authors.forename: Adele
authors.initials: A
authors.lastname: Zanetti
authors.forename: Gabriella
authors.initials: G
authors.lastname: Moroni
authors.forename: Carlo
authors.initials: C
authors.lastname: Salvarani
authors.forename: Mario
authors.initials: M
authors.lastname: Savi
authors.forename: Carlo
authors.initials: C
authors.lastname: Buzio
authors.forename: Tauro M
authors.initials: TM
authors.lastname: Neri
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D001327:Autoimmune Diseases / Q000235:genetics* / Q000276:immunology*; D002648:Child; D015331:Cohort Studies; D005260:Female; D020022:Genetic Predisposition to Disease; D005838:Genotype; D015234:HLA-A Antigens / Q000235:genetics; D015235:HLA-B Antigens / Q000235:genetics; D006684:HLA-DR Antigens / Q000235:genetics*; D059811:HLA-DRB1 Chains; D006801:Humans; D008297:Male; D008875:Middle Aged; D020641:Polymorphism, Single Nucleotide; D012185:Retroperitoneal Fibrosis / Q000235:genetics* / Q000276:immunology*
chemical_list: D015234:HLA-A Antigens; D015235:HLA-B Antigens; D006684:HLA-DR Antigens; D059811:HLA-DRB1 Chains