Background/Purpose: Antineutrophil antibody-associated vasculitis (AAV) has been most extensively described and studied in non-African American populations. Little is known about the characteristics and outcomes of AAV in African American patients.

Methods: Retrospective chart review of patients self-identified as African-American with positive antineutrophil cytoplasmic antibody testing was performed. Records were reviewed in detail to establish disease classification of patients using the revised Chapel Hill 2012 AAV criteria.

Results: 21 patients with definite AAV were identified (10 GPA, 8 MPA, 3 with isolated renal AAV). Fourteen (66.7%) were female, with a mean age at symptom onset of 59 years. 4 patients (19%) were C-ANCA/PR-3 positive, all of whom had GPA. 100% of isolated renal AAV and 75% of MPA patients were P-ANCA/MPO positive. Nine patients (42.8%) were antinuclear antibody positive, and 5 patients (23.8%) had a positive rheumatoid factor. At the time of diagnosis for the 18 GPA and MPA patients, recurrent sinusitis was found in 12 patients (66.7%), but otalgia or otitis was less common, noted in only 4 patients (22.2%) Sixteen patients (88.9%) had pulmonary infiltrates, ten patients (55.6%) had diffuse alveolar hemorrhage, and 8 patients (44.4%) required mechanical ventilation at presentation. 11 patients (52.3%) of the entire cohort required hemodialysis at presentation, and 12 patients (57.1%) ultimately developed end stage renal disease (ESRD). Fourteen patients had concomitant hypertension, with a mean number of antihypertensive medications of 1.7. Seven patients had diabetes mellitus, with a mean glycated hemoglobin of 7.5 and mean duration of disease of 7.7 years. 9 patients (42.8%) developed at least one relapse, with a mean time to relapse of 20.5 months following diagnosis. Eight patients (38%) died, with a mean time to death from diagnosis of 32 months.

Conclusion: African-American patients with AAV frequently present with pulmonary and renal manifestations, which are often severe at onset, requiring supportive care. More than half of this cohort went on to develop ESRD, and over one-third died on average within 3 years following their diagnosis, demonstrating that AAV may have an aggressive course in these patients. There are many possible factors that could influence these outcomes, including other comorbid conditions, delay in diagnosis, genetics, environmental factors, and limited access to care. Further study is needed to better understand factors that influence AAV severity and course in this population in order to improve long-term outcomes and survival.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/disease-characteristics-and-outcomes-in-african-american-patients-with-antineutrophil-cytoplasmic-antibody-associated-vasculitis-a-high-risk-group-for-poor-outcomes/