Kidney Week

Abstract: SA-PO833

Collaborative Study: Identifying Risk Factors for ESKD in Leukocyte Chemotactic Factor 2 (ALECT2)-Associated Amyloidosis

Session Information

• C3G, TMA, MGRS, Amyloidosis, and More
  October 26, 2024 | Location: Exhibit Hall, Convention Center
  Abstract Time: 10:00 AM - 12:00 PM

Category: Glomerular Diseases

• 1402 Glomerular Diseases: Clinical, Outcomes, and Therapeutics

Authors

• Vargas-Brochero, Maria Jose, Mayo Clinic Minnesota, Rochester, Minnesota, United States

Maria Jose Vargas-Brochero, MD, MS, MSc

• Vaughan, Lisa E., Mayo Clinic Minnesota, Rochester, Minnesota, United States

Lisa E. Vaughan, MS

• Zavala Miranda, María Fernanda, Instituto Nacional de Ciencias Medicas y Nutricion Salvador Zubiran, Ciudad de Mexico, Ciudad de México, Mexico

María Fernanda Zavala Miranda

• Uribe-Uribe, Norma O., Instituto Nacional de Ciencias Medicas y Nutricion Salvador Zubiran, Ciudad de Mexico, Ciudad de México, Mexico

Norma O. Uribe-Uribe, MD

• Mejia-Vilet, Juan M., Instituto Nacional de Ciencias Medicas y Nutricion Salvador Zubiran, Ciudad de Mexico, Ciudad de México, Mexico

Juan M. Mejia-Vilet, MD, MS, PhD

• Leung, Nelson, Mayo Clinic Minnesota, Rochester, Minnesota, United States

Nelson Leung, MD

Background

ALECT2 represents the third type of kidney-involved amyloidosis; its clinical prognosis and natural history are uncertain.

Methods

This was a retrospective cohort study of adults diagnosed with kidney biopsy-proven ALECT2 by immunohistochemistry or mass spectrometry between 1/1/2000 and 12/31/2022 at two institutions. Patients with end-stage kidney disease (ESRD) at diagnosis or without follow-up were excluded. ESRD-free survival was estimated using the Kaplan-Meier method. Cox regression was used to evaluate associations between characteristics at diagnosis and risk of incident ESRD

Results

There were 54 patients meeting inclusion criteria for this study (mean (SD) age 63.7 (7.2) years, 28 (51.8%) male and 48 (88.8%) Hispanic. There were a total of 19 (35.2%) ESRD events occurring during a mean (SE) follow-up of 7.1 (0.8) years. Estimated survival was 92% (95%CI: 85%-99%) at one year, 79% (95%CI: 67%-92%) at two years, and 70% (95%CI: 55%-88%) at 5-year follow-up. Patients with a higher percentage of glomerulosclerosis, serum creatinine, proteinuria, interstitial fibrosis/tubular atrophy, and a lower eGFR had a significantly higher risk of incident ESRD (P<0.05 for all; see Table 1)

Conclusion

This study reports long-term renal survival for ALECT2 patients and identifies several promising biomarkers associated with worse renal survival