028 Patient genetics shape the autoimmune response in pemphigus vulgaris

Pemphigus Vulgaris (PV) is a rare autoimmune blistering disease with multifactorial origin. Key Human Leukocyte Antigen (HLA) alleles, including DRB1*0402 and DQB1*0503, have been linked to the development of PV. However, it not known how HLA types vary ethnicity they shape autoimmunity in We assessed correlations between factors genotype, ethnicity, auto-antibody levels, lesion distribution cohort 293 patients. Eighty-one percent patients typed as either or DQB1*0503 high prevalence Ashkenazi Jewish Caucasian (non-Jewish) descent Southeast Asian descent. Patients typing had higher levels anti-desmoglein (Dsg)3 antibodies than without mucosal only lesions more often cutaneous mucocutaneous lesions. anti-Dsg1 compared other groups rates types. also report an unexpected association DRB1*0804 PV African descent, which 64% carried allele. was rarely seen ethnicities. positive presented highly elevated anti-Dsg3. neither heritage nor correlated predilection any specific morphology. A group that DRB1*0402, lowest anti-Dsg3 highest rate solely carriers these alleles. These data show impact genetic on response. It important note though are strict; patient can present morphology antibody profile. Nevertheless, our study advances goal elucidating mechanisms determine activity, knowledge necessary identify individuals at risk, predict prognosis, eventually achieve individually tailored medical therapies.