4.6 Article

Renal Involvement in IgG4-Related Disease: From Sunlight to Twilight

Journal

FRONTIERS IN MEDICINE
Volume 8, Issue -, Pages -

Publisher

FRONTIERS MEDIA SA
DOI: 10.3389/fmed.2021.635706

Keywords

IgG4-related disease; tubulointerstitial nephritis (TIN); retroperitoneal fibrosis; membranous nephropathy; ANCA; associated vasculitis

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IgG4-Related Disease primarily affects the kidneys, potentially leading to tubulointerstitial nephritis or membranous nephropathy. While responsive to steroids, in some cases it can still result in permanent kidney damage.
IgG4-Related Disease (IgG4-RD) is a fibroinflammatory condition characterized by a typical histopathological pattern (dense lymphoplasmacytic infiltrate with prevalent IgG4+ plasma cells and storiform fibrosis), which may involve the kidney both directly (IgG4-related kidney disease, IgG4-RKD) or indirectly, as a consequence of post-renal ureteral obstruction due to retroperitoneal fibrosis (IgG4-RD RF). The most frequent presentation of IgG4-RKD is IgG4-related tubulointerstitial nephritis (TIN), but a glomerular disease can be present, in most of the cases a membranous nephropathy. Albeit steroid-responsive, in some cases renal manifestations may lead to progressive and permanent organ damage. In this review we describe four clinical cases representative of typical and less typical renal manifestations of IgG4-RD, emphasizing a potential, subclinical, early involvement of the kidney in the disease.

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