Sodium-glucose cotransporter 2 inhibitors reduce albuminuria in patients with Fabry disease: a real-world case series.

Abstract

Background: Fabry disease is a rare X-linked multisystem disease, with progressive proteinuric kidney disease contributing significantly to morbidity and mortality of these patients. Evidence shows that sodium-glucose cotransporter 2 inhibitors (SGLT2Is) can reduce proteinuria and slow progression to end-stage kidney disease in both diabetic and non-diabetic kidney disease.

Aim: Evaluate the effects of SGLT2I on kidney function and albuminuria in patients with Fabry disease.

Methods: Single-centre real-world case series reviewing electronic medical records of patients with Fabry disease who initiated therapy with dapagliflozin or empagliflozin (n = 11). Changes in urine albumin-creatinine ratio (uACR) and creatinine before and after treatment with SGLT2I were analysed using Wilcoxon signed-rank test. Two-tailed P-values <0.05 were considered significant.

Results: Eleven patients were followed for up to 19 months after commencement of SGLT2I. An overall significant reduction in albuminuria (P = 0.05) was seen with SGLT2I use in the Fabry cohort. Median uACR before SGLT2I was 76 mg/mmol (interquartile range (IQR) 47-141) and after SGLT2I was 39 mg/mmol (IQR 18-95) (P = 0.05). All patients with uACR >100 mg/mmol had reduction in albuminuria over the study period. SGLT2Is were well tolerated overall, with only one case resulting in cessation of treatment due to adverse effects.

Conclusion: These results suggest SGLT2Is can significantly reduce albuminuria in a portion of patients with Fabry-related kidney disease and offer additional treatment for Fabry nephropathy. Given the nature of the study design and small case numbers, further long-term controlled studies are required to evaluate the long-term efficacy of this medication class in both cardiac and renal outcomes in Fabry disease.