Renal involvement in IgG4-related disease

Jean-Jacques Boffa (1) , Emmanuel Esteve (1) , David Buob (1)

1 Des Maladies Rénales Rares aux Maladies Fréquentes, Remodelage et Réparation

IgG4-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.

Domaines: Sciences du Vivant [q-bio]

Jean-Jacques Boffa, Emmanuel Esteve, David Buob. Renal involvement in IgG4-related disease. La Presse Médicale, 2020, 49 (1), pp.1-6. ⟨10.1016/j.lpm.2020.104017⟩. ⟨hal-03490381⟩