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

Frontiers in Medicine

Ilaria Puxeddu¹  Riccardo Capecchi¹  Antonio G. Tavoni¹  Federico Pratesi¹  Paola Migliorini¹  Cristina Croia¹  Diego Moriconi²  Domenico Giannese³  Maria F. Egidi³  Angelo G. Bonadio⁴ 

[1] Clinical Immunology and Allergy Unit, Department of Clinical and Experimental Medicine, University of Pisa, Pisa, Italy;Department of Clinical and Experimental Medicine, University of Pisa, Pisa, Italy;Department of Surgical, Medical and Molecular Pathology and Critical Care Medicine, University of Pisa, Pisa, Italy;Nephrology, Dialysis and Transplantation Unit, Azienda Ospedaliero Universitaria Pisana, Pisa, Italy;Pathology Unit, University of Pisa, Pisa, Italy;

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

DOI  :  10.3389/fmed.2021.635706

来源: Frontiers

【 摘 要 】

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.

【 授权许可】

CC BY