Background/Purpose: Henoch-Schönlein purpura (HSP) is essentially a childhood disease, being the most common type of vasculitis in children and an infrequent condition in adults. An increased familial occurrence supports a genetic predisposition for this vasculitis. In this context, the role of the HLA (human leukocyte antigen) region in the HSP pathogenesis has previously been studied. However, data reported so far on the potential association of HSP with HLA-DRB1 alleles are scarce and they were generally the result of small series of HSP patients with often contradictory results. To further investigate whether HLA-DRB1alleles are implicated in the susceptibility and severity of HSP, we performed a study that encompassed the largest series of HSP patients ever assessed for genetic studies in Caucasian individuals.
Methods: Our study population included 279 Spanish patients diagnosed with HSP and 335 sex and ethnically matched controls. HSP patients fulfilling the American College of Rheumatology (Arthritis Rheum 1990; 33: 1114-21) and the Michel et al (J Rheumatol 1992; 19: 721-8) classification criteria were recruited from Hospital Universitario Lucus Augusti (Lugo), Hospital Universitario Marqués de Valdecilla (Santander), Hospital Universitario La Princesa (Madrid), Hospital Universitario San Cecilio (Granada) and Hospital Universitario de Basurto (Bilbao). HLA-DRB1 phenotypes were determined using PCR-SSOP Luminex.
Results: After adjusting the results for multiple testing corrections, we disclosed a statistically significant increased frequency of HLA-DRB1*01 in HSP patients compared to controls (p<0.001, OR=3.98 [95% CI: 2.68-5.95]). In contrast, a significantly decreased frequency of HLA-DRB1*03 was observed in HSP patients compared to controls (p<0.001, OR=0.18 [95% CI: 0.085-0.37]). When patients were stratified according to the presence of nephritis or gastrointestinal manifestations, we disclosed that although no specific HLA-DRB1 association with nephritis was observed, HLA-DRB1*07was significantly reduced in the group of HSP patients who experienced gastrointestinal manifestations (p=0.0011, OR=0.36 [95% CI: 0.19-0.71]) even after adjusting the results for multiple testing correction (p=0.012).
Conclusion: Our study supports an association of HSP with HLA-DRB1*01. In contrast, a protective effect against the development of HSP was observed in individuals carrying the HLA-DRB1*03 allele. HLA-DRB1*07exerts a protective effect against the development of gastrointestinal manifestations in patients with HSP.
This study was supported by European Union FEDER funds and a grant from “Fondo de Investigaciones Sanitarias” (PI12/00193) (Spain). RLM is a recipient of a Sara Borrell postdoctoral fellowship from the Instituto de Salud Carlos III at the Spanish Ministry of Health (Spain) (CD12/00425). FG and BU are supported by funds from the RETICS Program (RIER) (RD12/0009/0013) (Spain).
Disclosure:
R. López-Mejías,
None;
F. Genre,
None;
B. Sevilla Pérez,
None;
S. Castañeda,
None;
N. Ortego-Centeno,
None;
J. Llorca,
None;
B. Ubilla,
None;
T. Pina Murcia,
None;
V. Calvo-Rio,
None;
A. Márquez,
None;
L. Sala-Icardo,
None;
J. A. Miranda-Filloy,
None;
M. Conde-Jaldón,
None;
L. Ortiz-Fernández,
None;
J. María Blanco-Madrigal,
None;
E. Galindez-Agirregoikoa,
None;
F. González Escribano,
None;
J. Martin,
None;
R. Blanco,
None;
M. González-Gay,
None.
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ACR Meeting Abstracts - https://acrabstracts.org/abstract/hla-drb101-is-associated-with-henoch-schonlein-purpura-in-the-spanish-population/