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Spontaneously Resolving Pancreatic IgG4-Related Disease Representing With Systemic Involvement and Membranous
Iwan G A Raza1, Stuart W Smith1, Dimitrios Chanouzas1,2
1Department of Renal Medicine, Queen Elizabeth Hospital, Birmingham, United Kingdom.
Immunoglobulin G4-related disease (IgG4-RD) can cause secondary membranous nephropathy (MN) without kidney inflammation. This case highlights IgG4-RD presenting as nephrotic syndrome, successfully treated with steroids and rituximab.
Area of Science:
- Nephrology
- Immunology
- Gastroenterology
Background:
- Secondary membranous nephropathy (MN) is increasingly linked to immunoglobulin G4 (IgG4)-related disease (IgG4-RD).
- MN in IgG4-RD typically co-occurs with tubulointerstitial nephritis but can manifest independently.
- IgG4-RD is a multisystem fibroinflammatory condition characterized by IgG4-producing plasma cell infiltration.
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