Abstract: FR-PO0782
ANCA-Associated Vasculitis in Two Family Members After Silica Exposure
Session Information
- Glomerular Diseases: ANCA Vasculitis, Anti-GBM Disease, and Crescentic GN
October 23, 2026 | Location: Exhibit Hall A, Convention Center
Abstract Time: 10:00 AM - 12:00 PM
Category: Glomerular Diseases
- 1402 Glomerular Diseases: Clinical, Outcomes, and Therapeutics
Authors
- Basit, Muhammad Abdul, United Health Services Hospitals Inc, Binghamton, New York, United States
- Quasem, Mohammad A., United Health Services Hospitals Inc, Binghamton, New York, United States
- Suo, Liye, SUNY Upstate Medical University, Syracuse, New York, United States
- Jayaraman, Venkatesh, United Health Services Hospitals Inc, Binghamton, New York, United States
- Ahmed, Waqar, United Health Services Hospitals Inc, Binghamton, New York, United States
- Choudhry, Zaima Zaeem Ul Hameed, United Health Services Hospitals Inc, Binghamton, New York, United States
Introduction
ANCA associated vasculitis (AAV) is a group of disorders caused by inflammation affecting multiple organs. Etiology not known, environmental exposure and genetic predisposition may be responsible.
Here we present two brothers with AAV following exposure to slica dust.
Case Description
62-year-old male presented with decreased appetite, weakness, weight loss. Worked in stone cutting for many years. CT chest suggestive of pulmonary silicosis Urinalysis; positive for protein/blood. Urine protein/creatinine ratio 1.80.Serological studies p-ANCA positive, Myeloperoxidase ab titer 6.9 (normal <0.4), antiproteinase 3 ab, neg, <0.2, ANA titer neg, <2, C3/C4 negative.Renal biopsy chronic/active:crescentic glomerulonephritis with advanced chronicity, >50% interstitial fibrosis and tubular atrophy (image 1)
His brother also worked in stone cutting, has pumonary silicosis and AAV..
Discussion
AAV are immune-mediated disorders in which tissue injury results from interplay of an initiating inflammatory event and highly specific immune response against neutrophil granule proteins leading to high titers of autoantibodies (ANCA).These autoantibodies produce tissue damage via interactions with neutrophils and endothelial cells.
The events leading to the initiation of AAV are not well understood. Environmental exposure may serve as an adjuvant in patients with a genetic predisposition to AAV.. Our patients had silica dust exposure (stone cutting) for many years.
As our patients (two brothers), family clusters of AAV have been described.
Genome-wide association studies (GWAS) have identified a number of genes associated with susceptibility to AAV.
A large cohort of patients with AAV in Europe revealed associations between patients with proteinase 3 (PR3)-ANCA serotype and genes encoding alpha-1 antitrypsin (SERPINA1, the endogenous inhibitor of PR3) and human leukocyte antigen (HLA)-DP. Anti-myeloperoxidase (MPO)-ANCA was associated with HLA-DQ. .
Further study is needed to better understand the genetic factors involved in familal clusters of AAV.
Renal biopsy: chronic active crescentic glomerulonephritis with vasculitis