Introduction: A study was conducted to determine whether the human leukocyte antigen (HLA) B alleles are implicated in the susceptibility to Henoch-Schönlein purpura (HSP) in the largest series of Caucasian HSP patients ever assessed for genetic studies.
Methods: The study population was composed of 349 Spanish patients diagnosed with HSP fulfilling the American College of Rheumatology and the Michel et al. classification criteria, and 335 sex and ethnically matched controls. HLA-B phenotypes were determined by sequencing-based typing (SBT) and analyzed by chi-square or Fisher exact test.
Results: A statistically significant increase of HLA-B*41:02 allele in HSP patients when compared with controls was found (8.3% versus 1.5% respectively; P = 0.0001; OR (odds ratio) =5.76 [2.15-19.3]). These results remained statistically significant after adjusting for Bonferroni correction (P = 0.0028). An internal validation also confirmed the susceptibility effect on HSP associated with HLA-B*41:02 (OR = 5.70 [1.98-16.44]). Since a former study described an association between HLA-DRB1*01:03 and HSP susceptibility, we also evaluated the implication of HLA-B*41:02 independently of HLA-DRB1*01:03. Interestingly, the association remained statistically significant (P = 0.0004, OR = 4.97 [1.8-16.9]). No HLA-B association with specific HSP clinical features was found.
Conclusions: Our study indicates that HLA-B*41:02 is associated with the susceptibility to HSP in Spanish patients irrespective of HLA-DRB1 status.
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http://dx.doi.org/10.1186/s13075-015-0622-5 | DOI Listing |
Cureus
December 2024
Department of Respiratory Medicine, Juntendo University School of Medicine Graduate School of Medicine, Tokyo, JPN.
IgA vasculitis (IgAV) generally occurs in young people and presents with a tetrad of symptoms: purpura, abdominal pain, arthralgia, and nephritis. However, it may have an atypical course without the typical tetrad. Diffuse alveolar hemorrhage (DAH), heart failure, and stroke are known complications of IgAV but are all very rare.
View Article and Find Full Text PDFCureus
December 2024
Nephrology, Ibn Sina Hospital, Rabat, Rabat, MAR.
Cryoglobulinemic vasculitis is a rare small-vessel vasculitis leading to multi-organ dysfunction, often associated with chronic infections like hepatitis C virus (HCV), and autoimmune disorders. Most cases involve mixed monoclonal or polyclonal immunoglobulins, presenting symptoms such as purpura, arthralgias, and weakness. Severe organ involvement, particularly cardiac, is rare but potentially life-threatening.
View Article and Find Full Text PDFXi Bao Yu Fen Zi Mian Yi Xue Za Zhi
January 2025
Hematologic Disease Center, First Affiliated Hospital of Xinjiang Medical University, Xinjiang Uygur Autonomous Region Research Institute of Hematology, Xinjiang Medical University, Urumqi, Xinjiang Uygur Autonomous Region, Wulumuqi 830011, China. *Corresponding author, E-mail:
Objective This study investigated the regulatory effect of high mobility group protein B1 (HMGB1) in the peripheral blood of patients with primary immune thrombocytopenia (ITP) on myeloid dendritic cells (mDC) and Th17/regulatory T cells (Treg) balance. Methods The study enrolled 30 newly diagnosed ITP patients and 30 healthy controls.Flow cytometry was used to measure the proportion of mDC, Th17, and Treg cells in the peripheral blood of ITP patients and healthy controls.
View Article and Find Full Text PDFRheumatology (Oxford)
January 2025
Department of Immunology and Rheumatology, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico.
Objectives: To evaluate the prevalence and clinical associations of anti-C1q antibodies in IgG4-related disease (IgG4-RD), focusing on renal involvement and cutaneous small-vessel vasculitis (CSVV).
Methods: We enrolled patients who met the revised 2020 Comprehensive Diagnostic Criteria and/or the 2019 ACR/EULAR Classification Criteria for IgG4-RD. Variables included demographics, organ involvement, clinical phenotypes, disease activity, serum biomarkers, follow-up duration, remission, and relapses.
Sci Rep
January 2025
Discovery3 Team, Department of Research and Early Development, GC Biopharma, 93, Ihyeon-ro 30Beon-gil, Giheung-gu, Yongin-si, Gyeonggi-do, South Korea.
Immune-mediated thrombotic thrombocytopenic purpura (iTTP) is a rare and life-threatening blood disorder characterized by the formation of blood clots in small blood vessels. It is caused by antibodies targeting the A disintegrin and metalloprotease with thrombospondin type 1 repeats, member 13 (ADAMTS13), which plays a role in cleaving von Willebrand factor. Most patients with iTTP have autoantibodies against specific domains of the ADAMTS13 protein, particularly the cysteine-rich and spacer domains.
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