Korean J Med.  2014 Jul;87(1):96-100.

IgG4-Related Tubulointerstitial Nephritis Accompanied by Henoch-Schonlein Purpura

• Yang H
• Choi SK
• Kim B
• Yoo JY
• Koh ES
• Chang YS
• Chung S

Abstract

Tubulointerstitial nephritis is one of the common manifestations of immunoglobulin G (IgG)4-related disease; however, among all cases of tubulointerstitial nephritis undergoing renal biopsies, IgG4-related tubulointerstitial nephritis seems to be relatively rare because of its trivial urinary findings. A previously healthy 54-year-old man was referred to our clinic with a 4-week history of lower leg purpura and renal dysfunction. A kidney biopsy was planned because of bilateral renomegaly, by imaging studies, and elevated serum creatinine levels. Pathological findings in the kidney showed prominent infiltration of IgG4-postive plasma cells in the tubulointerstitium, but not the glumeruli. A skin biopsy revealed leukocytoclastic vasculitis, accompanied by deposition of IgA and C3 in the vascular wall, indicating Henoch-Schonlein purpura (HSP). Although cases of combined IgG4-related disease and microvasculitis, including HSP, are extremely rare, the possibility of an association between two diseases deserves attention.

Keyword

Immunoglobulin G4; Nephritis, Interstitial; Henoch-Schonlein purpura

MeSH Terms

Biopsy
Creatinine
Humans
Immunoglobulin A
Immunoglobulin G
Kidney
Leg
Middle Aged
Nephritis, Interstitial*
Plasma Cells
Purpura
Purpura, Schoenlein-Henoch*
Skin
Vasculitis
Creatinine
Immunoglobulin A
Immunoglobulin G