Ms YI ZHANG1, Prof. HUI XU1
1Xiangya Hospital Of Central South University, Changsha, China
Biography:
A postgraduate from Xiangya Hospital, Central South University, I work under the guidance of Prof. Xu Hui, Chief of Nephrology Department, with research emphasis on IgA-associated disorders.
Abstract:
Introduction: Rapidly progressive IgA nephropathy (RPIgAN) is a severe phenotype defined by a ≥50% decline in eGFR within three months, often leading to end-stage kidney disease. Current treatments relying on corticosteroids and immunosuppressants have limited application due to toxicity risks. Telitacicept, a dual-target fusion protein inhibiting BLyS and APRIL, reduces autoantibody production. We report the preliminary efficacy of Telitacicept in treating RPIgAN.
Case Presentation: We present five cases treated with Telitacicept (160 mg/week), either as monotherapy or combined with cyclophosphamide/budesonide. Patient 1 (24M) achieved clinical remission by week 16, with serum creatinine (SCr) improving from 193.6 to 100.3 μmol/L and UPCR dropping to 0.12 g/g. Patient 2 (41M) maintained stable renal function and achieved negative proteinuria on monotherapy. Patient 3 (30F) showed renal recovery (SCr 200.0 to 155.0 μmol/L) and resolution of proteinuria. Patient 4 (26M), presenting with severe impairment, achieved SCr stabilization and significant proteinuria reduction (5.13 to 1.09 g/d). Patient 5 (29F) overcame refractory proteinuria, decreasing from 3.57 to 0.98 g/d after five months.
Discussion: Proteinuria reduction is critical for prognosis in IgAN. Telitacicept effectively lowers proteinuria by inhibiting B-cell maturation and reducing galactose-deficient IgA1 production. The consistent improvement in renal metrics across these cases highlights its potential utility in high-risk patients.
Conclusion: Telitacicept demonstrated favorable efficacy and safety in this case series. It represents a promising therapeutic strategy for RPIgAN by stabilizing renal function and reducing proteinuria.