Case report: Sequential complement inhibition and BAFF/APRIL blockade in progressive IgA nephropathy and IgA vasculitis nephritis: a report of two cases - Report - MDSpire

Case report: Sequential complement inhibition and BAFF/APRIL blockade in progressive IgA nephropathy and IgA vasculitis nephritis: a report of two cases

  • By

  • Xinyuan Tian

  • Wanyin Hou

  • Xiaojuan Yu

  • Lei Jiang

  • Xin Zhang

  • Xu Zhang

  • Suxia Wang

  • Ying Tan

  • Lijun Liu

  • July 8, 2026

  • 0 min

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Clinical Report: Combined complement inhibition and BAFF/APRIL targeting in advanced IgA nephropathy and IgA vasculitis nephritis

Background

Rapidly progressive IgAN and IgAVN are associated with severe renal impairment and poor outcomes. Both conditions share pathogenic mechanisms, including the deposition of galactose-deficient IgA1 and complement system activation. Current treatment strategies remain uncertain.

Data Highlights

No numerical data is available in the source material.

Key Findings

  • Both patients had rapidly progressive crescentic disease with significant proteinuria.
  • Sequential therapy included eculizumab, iptacopan, and telitacicept.
  • After treatment, one patient showed a decrease in proteinuria from 5.72 g/day to 1.55 g/day.
  • Both patients experienced stabilization of kidney function during follow-up.

Clinical Implications

The cases presented indicate that multi-pathway targeted therapy may be considered for patients with aggressive forms of IgAN and IgAVN.

Related Resources & Content

  1. KDIGO 2025 Clinical Practice Guideline for the Management of Immunoglobulin A Nephropathy (IgAN) and Immunoglobulin A Vasculitis (IgAV)
  2. Alternative Complement Pathway Inhibition with Iptacopan in IgA Nephropathy | New England Journal of Medicine
  3. Clinical Rheumatology — Interstitial Nephritis in ANCA-Associated Vasculitis Without Glomerulonephritis: A Case Series and Review of the Literature
  4. Clinical Rheumatology — Distribution and Presence of FOXP3 + Cells in Kidney Biopsies from Patients with Lupus Nephritis and ANCA-Associated Vasculitis
  5. Clinical Rheumatology — A Comprehensive Review of Potential Pharmacological Interventions for Nephritis in Pediatric Patients with IgA Vasculitis (HSP)
  6. Frontiers in Immunology — Recurrence of IgA nephropathy in a kidney transplant patient successfully treated with iptacopan—a case report
  7. KDIGO 2025 Clinical Practice Guideline for the Management of Immunoglobulin A Nephropathy (IgAN) and Immunoglobulin A Vasculitis (IgAV)
  8. KDIGO 2024 Clinical Practice Guideline for the Management of Antineutrophil Cytoplasmic Antibody (ANCA)–Associated Vasculitis
  9. Alternative Complement Pathway Inhibition with Iptacopan in IgA Nephropathy | New England Journal of Medicine
  10. New Novartis Fabhalta® (iptacopan) data show clinically meaningful and statistically significant proteinuria reduction of 38.3% versus placebo for patients with IgA nephropathy (IgAN) | Novartis United States of America
  11. FABHALTA (iptacopan) capsules, for oral use
  12. A Phase 2 Trial of Sibeprenlimab in Patients with IgA Nephropathy | New England Journal of Medicine
  13. A phase 2b, randomized, double-blind, placebo-controlled, clinical trial of atacicept for treatment of IgA nephropathy - ScienceDirect
  14. #812 Phase 2b ORIGIN study open label extension with atacicept in patients with IgA nephropathy and persistent proteinuria: week 72 interim analysis | Nephrology Dialysis Transplantation | Oxford Academic
  15. Efficacy, safety, and biomarker changes of B-cell activating factor and A proliferation-inducing ligand-targeted therapies in IgA nephropathy: a systematic review and meta-analysis of randomized controlled trials | Journal of Nephrology | Oxford Academic

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