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Abstract: PO1736

Single-Center Experience of Antineutrophil Cytoplasmic Autoantibody-Associated Vasculitis in a Region of Central Appalachia

Session Information

Category: Glomerular Diseases

  • 1202 Glomerular Diseases: Immunology and Inflammation

Authors

  • Aljuhani, Muhammad M., West Virginia University Health Sciences Center, Morgantown, West Virginia, United States
  • Makati, Devan, West Virginia University Health Sciences Center, Morgantown, West Virginia, United States
  • Hoff, Abigail, West Virginia University Health Sciences Center, Morgantown, West Virginia, United States
  • Chaudhary, Vishy, West Virginia University Health Sciences Center, Morgantown, West Virginia, United States
  • Pellegrino, Bethany S., West Virginia University Health Sciences Center, Morgantown, West Virginia, United States
  • Schmidt, Rebecca J., West Virginia University Health Sciences Center, Morgantown, West Virginia, United States
  • Kannabhiran, Dinesh, West Virginia University Health Sciences Center, Morgantown, West Virginia, United States
Background

Antineutrophil cytoplasmic autoantibody-associated vasculitis (AAV) is a rare disease with significant morbidity and mortality. Suspicions that our caseload of AAV might exceed the estimated 3 in 100,000 incidence estimates prompted us to investigate the characteristics of our patients with AAV.

Methods

A retrospective study of all patients diagnosed with AAV at our center prior to December 31, 2019 was performed. Patients were identified based on ICD10 (349), ICD 9 (1846) codes or diagnosis of a positive ANCA lab test (589). Charts were reviewed for demographic and clinical information. Incidence was estimated for the 10-year period being January 1, 2009 using population estimate.

Results

A total of 225 patients had a confirmed diagnosis of AAV of whom 114 were males (50.6%) and 111 females (49.4%). 94.7% were Caucasian, 2.2% African American and 2.2% Hispanics, reflective of our population. Most were older (50.2% >60 years). The kidneys(67.6%), lungs (42.7%) and ENT organs(30.2%) were most commonly involved. The predominant ANCA subtype was p-ANCA(52.3%), followed by c-ANCA (43%) and ANCA-negative (4.7%). p-ANCA was most common in patients with renal involvement (58.8%) and c-ANCA was most common in patients with ENT involvement (60%); p <0.01. Of those with renal involvement, 51 needed dialysis (33.6%), 47 of whom became dialysis-dependent (30.9%). Mortality was high in patients with kidney (32.2%) and lung involvement (30.2%) compared to those with ENT involvement (16.2%); p=0.04. Preliminary estimates suggest a regional incidence that may exceed that of other states.

Conclusion

In our population, p-ANCA was the predominant subtype and incidence estimates did not mirror those of other areas. These findings suggest that AAV may differ in subtype predominance and incidence by geographic setting.

Organ Involvement
Organ involved
(N of patients)
c-ANCA
N (%)
p-ANCA
N (%)
Neg ANCA
N (%)
Renal (152)59 (39.9%)87 (58.8%)2 (1.4%)
Lung (96)44 (46.8%)47 (50%)3 (3.2%)
Eyes (15)9 (60%)5 (33.3%)1 (9.1%)
ENT (68)45 (68.2%)15 (22.7%)6 (9.1%)
Skin (21)8 (38.1%)9 (42.9%)4 (19%)
Brain (19)10 (52.6%)7 (36.8%)2 (10.5%)
Heart (3)2 (66.7%)1 (33.3%)0 (0%)
GI (2)0 (0%)2 (100%)0 (0%)