IgG4-related kidney disease--an update.

Abstract

PURPOSE OF REVIEW:

IgG4-related disease (IgG4-RD) is a recently recognized systemic inflammatory disorder that can affect most organs/tissues such as sarcoidosis. The kidney is a frequently affected organ with tubulointerstitial nephritis (TIN), the representative lesion of IgG4-RD. This review focuses on the latest knowledge of IgG4-related kidney disease (IgG4-RKD).

RECENT FINDINGS:

A wide range of renal manifestations of IgG4-RD, that is TIN, membranous glomerulonephritis (MGN) and other glomerular lesions, and pyelitis, are collectively referred to as IgG4-RKD. Clinically, decreased renal function, or characteristic imaging findings such as multiple low-density lesions on contrast-enhanced computed tomography or diffuse thickening of the renal pelvic wall, are typical presenting features. Although a rapid response to corticosteroid therapy is a very important feature of IgG4-TIN, in cases in which renal function is moderately to severely decreased before therapy, only partial recovery of renal function is obtained.

SUMMARY:

TIN with characteristic imaging findings is a typical manifestation of IgG4-RKD in the interstitium, while MGN is a representative manifestation of the glomerular lesions. Although IgG4 is a central feature of IgG4-RD, the recent discovery of IgG4-negative IgG4-RD raises questions about the causative role of the IgG4 molecule in this context.

• Authors: Mitsuhiro Kawano, Takako Saeki
• Journal: Current opinion in nephrology and hypertension (Curr Opin Nephrol Hypertens) Vol. 24 Issue 2 Pg. 193-201 (Mar 2015) ISSN: 1473-6543 [Electronic] England
• PMID: 25594543 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't, Review)

Chemical References

• Immunoglobulin G

Topics

• Animals
• Autoimmune Diseases (complications, diagnosis, therapy)
• Diagnostic Imaging
• Humans
• Immunoglobulin G (immunology)
• Kidney (immunology, pathology)
• Kidney Diseases (diagnosis, immunology, therapy)
• Kidney Glomerulus (immunology)