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Abstract: SA-PO419

ANCA-Associated Vasculitis in a Predominantly Hispanic Population in the Western United States

Session Information

Category: Glomerular Diseases

  • 1203 Glomerular Diseases: Clinical, Outcomes, and Trials

Authors

  • Zhong, Yan, LAC USC Medical Center, Los Angeles, California, United States
  • Petrosyan, Nina, Keck School of Medicine, Los Angeles, California, United States
  • Carpenter, Philip M., University of Southern California, Los Angeles, California, United States
  • Ding, Li, University of Southern California, Los Angeles, California, United States
  • O'Neal, C-TAGME, Becky M., University of Southern California/LAC+USC Medical Center, Los Angeles, California, United States
  • Shan, Hui Yi, University of Southern California/LAC+USC Medical Center, Los Angeles, California, United States
Background

ANCA-associated vasculitis (AAV) is a group of rare systemic autoimmune diseases with significant geographic and ethnic differences in disease prevalence and severity. Both environmental factors and genetic susceptibility are thought to contribute to such differences. Most published data thus far have been collected from Caucasian and Asian patients. The aim of this study is to characterize the features of AAV in a specific cohort of patients, predominately of Mexican descent, who reside in the Western United States.

Methods

A retrospective review of all native kidney biopsies for patients above age of 18 years old that were performed at the Los Angeles County University of Southern California Hospital from 2008-2018 was done. 43 patients with AAV were identified. Based on pathology results, we classified these patients into focal, crescentic, mixed and sclerotic subtypes.

Results

Among the 43 patients, 38 were Hispanic and 20 of which were from Mexico. The mean age at diagnosis was 49.8 years old. Patients’ median serum creatinine on presentation was 2.4 mg/dl. 17 patients (39.5%) had kidney involvement alone, 5 patients (11.6%) with kidney and nasopharyngeal, 13 (30.2%) with kidney and lung, and 8 (18.6%) with kidney, lung and nasopharyngeal involvement. 30 patients (73.2%) were myeloperoxidase (MPO) positive and 10 (24.4%) were proteinase (PR3) positive. One patient was both MPO and PR3 positive. Pathological classification revealed 15 focal, 11 crescentic, 8 mixed, and 9 sclerotic subtypes. The majority (83%) of the patients received steroids with rituximab or steroids with cyclophosphamide. At 1 year of follow up, 7 (16.3%) patients relapsed, 12 (27.9%) patients had ESRD, and 4 (9.3%) patients died.

Conclusion

Our study showed that this predominately Mexican-American cohort residing in the Western United States from 2008-2018 presented with AAV at younger age and had worse 1-year renal survival as compared to data published on Asian and Caucasian populations. In addition, kidney involvement and MPO positivity dominated this cohort.