Chronic periaortitis and HLA–DRB103: Another clue to an autoimmune origin

• Published in: Arthritis and rheumatism Vol. 55; no. 1; pp. 126 - 130
• Main Authors: Martorana, Davide, Vaglio, Augusto, Greco, Paolo, Zanetti, Adele, Moroni, Gabriella, Salvarani, Carlo, Savi, Mario, Buzio, Carlo, Neri, Tauro M.
• Format: Journal Article
• Language: English
• Published: Hoboken Wiley Subscription Services, Inc., A Wiley Company 15.02.2006
• Subjects: Adolescent; Adult; Aged; Autoimmune disease; Autoimmune Diseases - genetics; Autoimmune Diseases - immunology; Child; Chronic periaortitis; Cohort Studies; Female; Genetic Predisposition to Disease; Genotype; HLA-A Antigens - genetics; HLA-B Antigens - genetics; HLA-DR Antigens - genetics; HLA-DRB1 Chains; HLA–DRB103; Humans; Male; Middle Aged; Polymorphism, Single Nucleotide; Retroperitoneal fibrosis; Retroperitoneal Fibrosis - genetics; Retroperitoneal Fibrosis - immunology
• ISSN: 0004-3591; 1529-0131
• DOI: 10.1002/art.21698

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%; χ2 = 15.50, P = 0.000084, corrected P [Pcorr] = 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%; χ2=11.12, P = 0.0008, Pcorr = 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 (Pcorr = 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 (Pcorr = 0.369). Conclusion 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.