Telitacicept monotherapy for refractory idiopathic membranous nephropathy: a case report and literature review

Abstract

Background: Patients with refractory membranous nephropathy (MN) face risks of progressive renal decline and end-stage renal disease (ESRD), with limited treatment efficacy. Telitacicept, a novel humanized recombinant fusion protein effective in lupus nephritis and immunoglobulin A nephropathy (IgAN), has few reports on its use in refractory MN.

Case presentation: In May 2023, an 82-year-old man was admitted to Shaoxing Second Hospital with bilateral lower extremity edema. A renal biopsy confirmed idiopathic membranous nephropathy (IMN). Standard therapies, including glucocorticoids (GC), cyclophosphamide (CYC), tacrolimus (TAC), and rituximab (RTX), were ineffective. He developed steroid-induced diabetes and acute renal failure during treatment. Complete proteinuria remission was achieved with telitacicept monotherapy. The patient is under ongoing clinical follow-up.

Conclusion: Telitacicept holds promise as a potential second-line therapy for refractory MN when conventional treatments prove ineffective. However, due to the current lack of robust evidence supporting its use in IMN, further research is warranted to establish its clinical efficacy and safety.

Keywords: PLA2R; case report; literature review; refractory idiopathic membranous nephropathy; telitacicept.

Figure 1

Patients with IMN showed glomeruli sclerosis, diffusely thickened capillary walls and diffuse ribbon-shaped vacuolar degeneration. (A) Hematoxylin–eosin staining showed diffuse thickening of the glomerular capillary walls (×400); (B) Periodic acid Schiff staining showed granular/vacuolar degeneration of renal tubular epithelial cells (×400); (C) Periodic acid silver methenamine staining showed diffuse “satin-like” and vacuolar degeneration of the glomerular basement membrane (×400); (D) Immunofluorescence staining showed diffuse fine granular PLA2R deposition along the capillary walls. (×200); (E) Electron microscopy showed scattered electron-dense deposits beneath the capillary basement membrane epithelium (×2,500), bar = 10 μm; (F) Electron microscopy showed scattered electron-dense deposits beneath the capillary basement membrane epithelium (×1,500), bar = 20 μm.

Figure 2

The timeline for diagnosis and treatment.