Kidney Week

Abstract: SA-PO641

Protective Effect of Ambrisentan on Proteinuria in Patients with Alport Syndrome

Session Information

• Genetic Kidney Diseases: Models, Mechanisms, and Therapies
  October 26, 2024 | Location: Exhibit Hall, Convention Center
  Abstract Time: 10:00 AM - 12:00 PM

Category: Genetic Diseases of the Kidneys

• 1202 Genetic Diseases of the Kidneys: Non-Cystic

Authors

• Song, Zhuoran, Peking University First Hospital Department of Nephrology Renal Division, Beijing, China

Zhuoran Song, MD

• Li, Yang, Peking University First Hospital Department of Nephrology Renal Division, Beijing, China

Yang Li, MD

• Zhang, Hong, Peking University First Hospital Department of Nephrology Renal Division, Beijing, China

Hong Zhang, MD, PhD

• Zhou, Xu-jie, Peking University First Hospital Department of Nephrology Renal Division, Beijing, China

Xu-jie Zhou, MD

Background

In recent years, endothelin receptor antagonists (ERAs) have stepped into the spotlight of chronic kidney disease treatment, showing a significant reduction in proteinuria and preservation of kidney function, such as SONAR study in diabetic nephropathy, DUPLEX trial in focal segmental glomerulosclerosis (FSGS) and PROTECT trial in IgA nephropathy. However, whether they are effective in Alport syndrome (AS) is unclear.

Methods

This was a real-world study. AS patients that had been treated with RAASi or RAASi combined with sodium-dependent glucose transporters 2 inhibitors (SGLT2i) / mineralocorticoid receptor (MRA) in stable doses for at least 3 months but still with proteiuria (＞0.5g/d）and eGFR＞25 ml/min/1.73m² were included. All of them received ambrisentan 5 mg daily. Efficacy was assessed by the changes in proteinuria. The safety outcomes included renal function change and tolerance.

Results

In total, 12 patients with AS were included. The mean baseline 24-hour urinary protein (UTP) (±SD) was 2.73 ± 1.56g/d and decreased to 2.14 ± 1.32g/d after two-month treatment (P=0.001). The mean eGFR was stable in the two months (mean 57.08 vs. 58.98 ml/min/1.73m² at baseline). 2 patients discontinued due to edema and 2 due to headache in the second month. Other patients never reported any side effects. Among the patients, four have been followed over 6 months with additional 4 months follow-up data. We observed a continued decrease trend in urinary protein, additional 20.85±10.67% from the second month in the UTP reduction, and 36.61±16.06% reduction from the baseline. The eGFR remained stable.

Conclusion

The pilot study suggests that introducing of ERA on top of RAASi with or without SGLT2i/MRA provides an additional antiproteinuric effect in AS.