Abstract: PO1611
ANCA-Associated Crescentic Glomerulonephritis (AAV-GN) in Patients with Chronic Lymphocytic Leukemia (CLL): A Case Series
Session Information
- Glomerular Diseases: Clinicopathological Features and Outcomes in IgAN, Lupus Nephritis, and Vasculitis
November 04, 2021 | Location: On-Demand, Virtual Only
Abstract Time: 10:00 AM - 12:00 PM
Category: Glomerular Diseases
- 1203 Glomerular Diseases: Clinical, Outcomes, and Trials
Authors
- Androga, Lagu A., Mayo Clinic Minnesota, Rochester, Minnesota, United States
- Alexander, Mariam P., Mayo Clinic Minnesota, Rochester, Minnesota, United States
- Casal Moura, Marta Isabel Rodrigues, Mayo Clinic Minnesota, Rochester, Minnesota, United States
- Rabe, Kari, Mayo Clinic Minnesota, Rochester, Minnesota, United States
- Parikh, Sameer, Mayo Clinic Minnesota, Rochester, Minnesota, United States
- Leung, Nelson, Mayo Clinic Minnesota, Rochester, Minnesota, United States
- Fervenza, Fernando C., Mayo Clinic Minnesota, Rochester, Minnesota, United States
- Kattah, Andrea G., Mayo Clinic Minnesota, Rochester, Minnesota, United States
Background
Previous case reports have identified an association between CLL and AAV-GN. However, information on the clinical and pathologic characteristics and long-term outcomes of AAV in the setting of CLL have not been well described.
Methods
We queried medical records and research databases of CLL and AAV subjects seen at our institution to identify patients with diagnoses of CLL and AAV-GN from 1990-2020. We analyzed patient demographics, AAV-GN, CLL specific characteristics, treatments, and outcomes. Kidney biopsies were also reviewed.
Results
We identified 12 patients with AAV-GN and CLL. The mean age at diagnosis was 65 years (48, 80) for CLL and 68 years (57, 80) for AAV-GN. 5 patients were diagnosed with CLL prior to AAV-GN, 4 the same month, and 2 developed CLL >3 years after AAV-GN. At the time of AAV-GN diagnosis, all had acute kidney injury, with a median serum creatinine (SCr) of 1.9mg/dL (SD 3.2). Other organs involved included lungs (n=3), skin (n=1), and eyes/ encephalitis (n=1). 9 patients p-ANCA-MPO and 2 had c-ANCA-PR3 and one with an indeterminate ANCA but had PR3. On light microscopy, all had crescents, no vasculitis of the arteries, but 9 patients had focal lymphoid infiltrates without a formal diagnosis of CLL in the kidneys. On immunofluorescence 6/12 had trace to 1+ of IgA, 5/12 with IgG and 4/12 with C3. 5/12 of the biopsies had mesangial deposits and majority (1 with diffuse and 7 with mild–moderate) had foot process effacement. All patients received treatment for AAV (9 with rituximab, and 3 with cytotoxic drugs). Renal outcomes were favorable with 11 patients showing an improvement or stabilization in SCr. One patient (p-ANCA MPO antibodies) developed end stage kidney disease, and 3 patients died, two from CLL and the other from heart failure.
Conclusion
In this case series of patients with CLL and AAV GN, the vast majority had had p-ANCA MPO, suggesting that the two conditions have either a common underlying lymphocyte dysfunction or that CLL is a predisposing factor to the development of AAV. Anti-CD20 monoclonal antibody therapy was most commonly used, and it led to remission of AAV-GN.