Drs. Martorana and Vaglio contributed equally to this work.
Chronic periaortitis and HLA–DRB1*03: Another clue to an autoimmune origin
Version of Record online: 6 FEB 2006
Copyright © 2006 by the American College of Rheumatology
Arthritis Care & Research
Volume 55, Issue 1, pages 126–130, 15 February 2006
How to Cite
Martorana, D., Vaglio, A., Greco, P., Zanetti, A., Moroni, G., Salvarani, C., Savi, M., Buzio, C. and Neri, T. M. (2006), Chronic periaortitis and HLA–DRB1*03: Another clue to an autoimmune origin. Arthritis & Rheumatism, 55: 126–130. doi: 10.1002/art.21698
- Issue online: 6 FEB 2006
- Version of Record online: 6 FEB 2006
- Manuscript Accepted: 1 AUG 2005
- Manuscript Received: 8 APR 2005
- Associazione Emma ed Ernesto Rulfo per la Genetica Medica
- Italian Ministry of Education, University and Research
- Chronic periaortitis;
- Retroperitoneal fibrosis;
- Autoimmune disease
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.
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.
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).
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.