• Jean-Jacques Boffa, Emmanuel Esteve, and David Buob.
• Inserm UMRS 1155, department of nephrology, Sorbonne université, hôpital Tenon, AP-HP, 75020 Paris, France. Electronic address: jean-jacques.boffa@aphp.fr.
• Presse Med. 2020 Apr 1; 49 (1): 104017.

AbstractIgG4-RD may affect several organs including kidneys. The kidney is involved in approximately 20% of patient with IgG4-RD. The most common intrinsic kidney disease is tubulointerstitial nephritis (IgG4-TIN). Retroperitoneal fibrosis (IgG4-RPF) may induce obstructive acute renal failure. More rarely, IgG4-RKD can manifest as a glomerular disease, in particular as a membranous nephropathy (MN). It mostly affects middle-aged to elderly men and causes acute or chronic renal dysfunction, multiple hypodense lesions on CT-Scan and various extra-renal lesions. Increased serum IgG4 and hypocomplementemia are the most important serological findings for the diagnosis of IgG4-RD and thus should be systematically assessed when IgG4-RKD is suspected. Specific diagnosis criteria for IgG4-TIN including interstitial infiltration of IgG4-positive plasma cells, storiform fibrosis and tubular basement membrane immune complex deposits have been proposed. Corticosteroids are effective and remain the first-line therapy but relapses or severe forms could respond to immunosuppressive therapy.Copyright © 2020 Elsevier Masson SAS. All rights reserved.

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