Chonnam Med J.  2015 Apr;51(1):39-42. 10.4068/cmj.2015.51.1.39.

IgG4-Related Systemic Disease Can Be Easily Mistaken as a Uroepithelial Tumor

• Han SY
• Lee SI
• Lee YH
• Kim AJ
• Lim HJ
• Ro H
• Chang JH
• Lee HH
• Chung W
• Jung JY

• KMID: 2172146
• DOI: http://doi.org/10.4068/cmj.2015.51.1.39

Abstract

Immunoglobulin G4-related disease (IgG4-RD) is a newly recognized systemic syndrome characterized by elevated serum IgG4 concentrations and tumefaction or tissue infiltration by IgG4-positive plasma cells. We experienced a case of IgG4-RD involving multiple organs in a 64-year-old female who was referred for a suspected uroepithelial tumor. A mass biopsy confirmed dense lymphoplasmacytic infiltration with an increased number of IgG4-positive plasma cells. We discuss this case and review the literature to bring IgG4-RD to the attention to clinicians because it responds dramatically well to steroid therapy and should be kept in mind as a differential diagnosis to avoid unnecessary surgery.

Keyword

Immunoglobulin G; Sclerosis; Kidney

MeSH Terms

Biopsy
Diagnosis, Differential
Female
Humans
Immunoglobulin G
Immunoglobulins
Kidney
Middle Aged
Plasma Cells
Sclerosis
Unnecessary Procedures
Immunoglobulin G
Immunoglobulins

Figure

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  FIG. 1 Immunoglobulin G4 (IgG4) levels before and after initiating prednisolone therapy. The IgG4 level was elevated before initiating steroid therapy and decreased subsequently with clinical improvement.

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  FIG. 2 (A) Coronary orbital computed tomography (CT) view. Arrows indicate bilateral lacrimal gland enlargement. (B) CT scan showing right lobectomy status and a low-density nodule in the left thyroid lobe. (C) Infiltrating soft tissue mass (arrows) involving both pelvocalyceal systems and extending to the left ureter level.

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  FIG. 3 Pelvocalyceal mass biopsy in a patient with immunoglobulin G4-related disease (IgG4-RD) shows plasma infiltration of lymphocytes (A, hematoxylin and eosin, ×400) and dense infiltration of IgG4-positive plasma cells (B, immunohistochemical stain; IgG4, ×400). Kidney biopsy showed relatively well-preserved glomeruli and tubulointerstitium (C, periodic acid Schiff, ×200).

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  FIG. 4 Computed tomography scan shows decreased extent of enlargement in both lacrimal glands (A) and both pelvocalyceal masses (B).

Reference

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