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IgG4-related nephropathy

Journal of Nephrology volume 29, pages 487–493 (2016)Cite this article

Abstract

IgG4-related disease (IgG4-RD) is a recently recognized disorder, often with multiple organ involvement, characterized by dense tissue infiltration of IgG4-positive plasma cells, storiform fibrosis, obliterative phlebitis and frequently elevated serum IgG4 concentration. The kidney can be involved either directly or indirectly. The most frequent direct renal manifestations of IgG4-RD are IgG4-related tubulointerstitial nephritis (TIN) and membranous glomerulonephropathy. Retroperitoneal fibrosis (RPF) is another condition that is frequently IgG4-related and that can indirectly affect the kidney causing ureteral obstruction and hydronephrosis. Contrast-enhanced computerized tomography, magnetic resonance imaging and 18F-fluorodeoxyglucose positron emission tomography/computed tomography show different imaging findings and are useful tools for monitoring therapeutic response. Steroid treatment is the first line of therapy, but relapsing or refractory forms of the disease are frequently observed and require more aggressive therapeutic approaches. At our centre, we treated three cases of aggressive IgG4-related TIN and two cases of IgG4-related RPF with an intensified, immune suppressive protocol, obtaining good results without severe adverse effects.

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Authors and Affiliations

  1. Nephrology and Dialysis Unit, Giovanni Bosco Hospital, Turin, Italy

    Giacomo Quattrocchio & Dario Roccatello

  2. Second level Master in Practical Rheumatology, University of Turin, Turin, Italy

    Giacomo Quattrocchio

  3. Department of Rare, Immunologic, Center of Research of Immunopathology and Rare Diseases (CMID), Hematologic and Immunohematologic Diseases, Giovanni Bosco Hospital, Turin, Italy

    Dario Roccatello

  4. University of Turin, Turin, Italy

    Dario Roccatello

Authors
  1. Giacomo Quattrocchio
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  2. Dario Roccatello
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Correspondence to Giacomo Quattrocchio.

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Cite this article

Quattrocchio, G., Roccatello, D. IgG4-related nephropathy. J Nephrol 29, 487–493 (2016). https://doi.org/10.1007/s40620-016-0279-4

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  • DOI: https://doi.org/10.1007/s40620-016-0279-4

Keywords

  • IgG4-related disease
  • IgG4-related kidney disease
  • Tubulointerstitial nephritis
  • Retroperitoneal fibrosis
  • Glucocorticoids
  • Rituximab