Membranous nephropathy associated with IgG4-related disease

Paolo Cravedi, Mauro Abbate, Elena Gagliardini, Miriam Galbusera, Simona Buelli, Ettore Sabadini, Maddalena Maras, Laurence H. Beck, David J. Salant, Ariela Benigni, Vivette D'Agati, Giuseppe Remuzzi

Research output: Contribution to journalArticle


Immunoglobulin G4 (IgG4)-related systemic disease is a rare condition characterized by high levels of circulating IgG4 and IgG4-positive plasma cell infiltrates in various organs, including the pancreas, salivary glands, biliary tract, liver, lung, and kidney. We describe a case of a 54-year-old man with IgG4-related systemic disease presenting with autoimmune pancreatitis and Mikulicz disease. Steroid therapy decreased circulating IgG4 levels and promoted regression of clinical signs. Thereafter, an increase in serum IgG4 values was followed by the occurrence of nephrotic-range proteinuria. Kidney biopsy showed membranous nephropathy with no IgG4-positive cell infiltrates. A search for circulating immune complexes was negative, and antibodies against M-type phospholipase A2 receptor could not be detected. Western blot analyses identified circulating IgG3 reacting with superoxide dismutase 2. This case suggests that membranous nephropathy represents an additional renal manifestation of IgG4-related systemic disease, with a pathogenesis possibly associated with neoproduction of autoantibodies targeting podocyte antigen(s).

Original languageEnglish
Pages (from-to)272-275
Number of pages4
JournalAmerican Journal of Kidney Diseases
Issue number2
Publication statusPublished - Aug 2011


  • immunoglobulin G4 (IgG4)-related disease
  • Membranous nephropathy
  • nephrotic syndrome
  • rituximab

ASJC Scopus subject areas

  • Nephrology

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  • Cite this

    Cravedi, P., Abbate, M., Gagliardini, E., Galbusera, M., Buelli, S., Sabadini, E., Maras, M., Beck, L. H., Salant, D. J., Benigni, A., D'Agati, V., & Remuzzi, G. (2011). Membranous nephropathy associated with IgG4-related disease. American Journal of Kidney Diseases, 58(2), 272-275.