Korean J Urol.  2013 Mar;54(3):209-211.

Immunoglobulin G4-Related Systemic Sclerosing Disease: A Case Involving the Ureter and Kidney

• Kim S
• Kim TG
• Choi SK
• Kim MJ
• Min GE
• Lee HL
• Yoo KH

Abstract

Immunoglobulin (Ig) G4-related sclerosing disease is a newly defined clinicopathological entity characterized by lymphoplasmacytic infiltration of IgG4-positive plasma cells and varying degrees of fibrosis within affected tissues. Patients usually exhibit multisystem involvement and often respond well to steroid and immunosuppressive therapy. This report presents a case of IgG4-related sclerosing disease involving the ureter and kidney. We hope to bring IgG4-related sclerosing disease to the attention of urologists, because it is an uncommon disease that commonly responds to systemic corticosteroids.

Keyword

Immunoglobulin G; Sclerosis; Ureter

MeSH Terms

Adrenal Cortex Hormones
Fibrosis
Humans
Immunoglobulin G
Immunoglobulins
Kidney
Plasma Cells
Sclerosis
Ureter
Adrenal Cortex Hormones
Immunoglobulin G
Immunoglobulins

Figure

• 

  FIG. 1 (A) Infiltrating soft tissue mass involving left pelvocalyceal system and extending to the level of left mid ureter, causing left hydronephrosis (arrow). (B) A small well-defined nodule in left lower lobe, enlarged left paraesophageal lymph node (arrows).

• 

  FIG. 2 (A) Periureteral mass biopsy. Hematoxylin and eosin stain shows fibrosis with lymphoplasmacytic aggregation (×400). (B) Periureteral mass biopsy. Immunoglobulin (Ig) G imunohistochemical staining shows more than 50 IgG4-positive cell/high-powered field (×400).

Reference

1. Neild GH, Rodriguez-Justo M, Wall C, Connolly JO. Hyper-IgG4 disease: report and characterisation of a new disease. BMC Med. 2006. 4:23.

2. Chari ST. Diagnosis of autoimmune pancreatitis using its five cardinal features: introducing the Mayo Clinic's HISORt criteria. J Gastroenterol. 2007. 42:Suppl 18. 39–41.

3. Lin YJ, Chen PC, Chen HA, Li CF. IgG4-related retroperitoneal fibrosis: the first reported case in a Chinese population. Int J Rheum Dis. 2010. 13:e70–e73.

4. Minato N, Takayama H, Mukai M, Miyagawa Y, Tsujihata M, Nonomura N, et al. A case report of retroperitoneal fibrosis associated with IgG4-related sclerosing disease. Hinyokika Kiyo. 2010. 56:371–375.

5. Kim KY, Kim DK, Woo SH, Kim ET, Lee SB. Laparoscopic partial nephrectomy: an useful method of decision making for determining the approach and surgical method based on the systematic classification of tumor location. Korean J Urol. 2008. 49:1067–1073.

6. Stone JR. Aortitis, periaortitis, and retroperitoneal fibrosis, as manifestations of IgG4-related systemic disease. Curr Opin Rheumatol. 2011. 23:88–94.

7. Van Moerkercke W, Verhamme M, Meeus G, Oyen R, Steenbergen WV. A case of IgG4-related sclerosing disease with retroperitoneal fibrosis, autoimmune pancreatitis and bilateral focal nephritis. Pancreas. 2009. 38:825–832.