Korean J Radiol.  2016 Feb;17(1):56-58. 10.3348/kjr.2016.17.1.56.

Appendiceal Immunoglobulin G4-Related Disease Mimicking Appendiceal Tumor or Appendicitis: A Case Report

• Kim HS
• Kang WK
• Chung DJ

• KMID: 2351163
• DOI: http://doi.org/10.3348/kjr.2016.17.1.56

Abstract

Immunoglobulin G4 (IgG4)-related disease is an autoimmune disease that forms tumorous lesions. Several cases involving various organs are reported, however, IgG4-related disease involving appendix has not been reported yet. In this report, we presented a case of IgG4-related disease of appendix, which raised a suspicion of appendiceal tumor or usual appendicitis and, therefore, led to unnecessary surgical resection. IgG4-related disease should be considered in the differential diagnosis for a mass-like swelling of the appendix, in order to avoid unnecessary surgery.

Keyword

IgG4-related disease; Appendix; CT

MeSH Terms

Appendiceal Neoplasms/*diagnosis
Appendicitis/*diagnosis
Appendix/immunology/*pathology
Autoimmune Diseases/*diagnosis/immunology
Diagnosis, Differential
Humans
Immunoglobulin G/*immunology
Male
Middle Aged
Neoplasms
Immunoglobulin G

Figure

• 

  Fig. 1 Immunoglobulin G4-related disease in 51-year-old man. A. Axial contrast-enhanced CT image shows 3.6 cm solid mass at appendiceal tip (arrows). Associated periappendiceal fat infiltrations suggest that mass is inflammatory. B. Axial contrast-enhanced CT taken 21 days after initial CT (A). No significant change in mass is noted. However, periappendiceal fat haziness is aggravated (arrows). C. Right hemicolectomy includes 29 cm of ascending colon and 21 cm of ileum. Appendix forms mass sized 4.3 × 2.8 × 1.7 cm with fat proliferations on surface (arrow). D. Hematoxylin-eosin staining shows plasmacytic infiltration in subserosa of appendix (black arrows). Mucosal layer and muscle layer of appendix (white arrows) are intact suggesting that main pathology lies in mesentery rather than appendix (× 12.5). E. Immunoglobulin G4 (IgG4) stain shows IgG4-positive plasmacytes. Brown pigmented cells (arrows) are IgG4-positive cells. On IgG staining, > 50% of IgG-positive cells are IgG4 positive (not presented) (× 400).

Reference

1. Stone JH, Zen Y, Deshpande V. IgG4-related disease. N Engl J Med. 2012; 366:539–551.

2. Brito-Zerón P, Ramos-Casals M, Bosch X, Stone JH. The clinical spectrum of IgG4-related disease. Autoimmun Rev. 2014; 13:1203–1210.

3. Coulier B, Montfort L, Beniuga G, Pierard F, Gielen I. Small bowel obstruction caused by peritoneal immunoglobulin g4-related disease mimicking carcinomatosis: case report. Korean J Radiol. 2014; 15:66–71.

4. Ko Y, Woo JY, Kim JW, Hong HS, Yang I, Lee Y, et al. An immunoglobulin G4-related sclerosing disease of the small bowel: CT and small bowel series findings. Korean J Radiol. 2013; 14:776–780.

5. Koizumi S, Kamisawa T, Kuruma S, Tabata T, Chiba K, Iwasaki S, et al. Immunoglobulin G4-related gastrointestinal diseases, are they immunoglobulin G4-related diseases? World J Gastroenterol. 2013; 19:5769–5774.

6. Lee H, Joo M, Song TJ, Chang SH, Kim H, Kim YS, et al. IgG4-related sclerosing esophagitis: a case report. Gastrointest Endosc. 2011; 73:834–837.

7. Lopes J, Hochwald SN, Lancia N, Dixon LR, Ben-David K. Autoimmune esophagitis: IgG4-related tumors of the esophagus. J Gastrointest Surg. 2010; 14:1031–1034.

8. Raina A, Yadav D, Regueiro M, Krasinskas AM, Saul MI, Sapienza DA, et al. Mucosal IgG4 cell infiltration in ulcerative colitis is linked to disease activity and primary sclerosing cholangitis. Inflamm Bowel Dis. 2013; 19:1232–1237.

9. Chetty R, Serra S, Gauchotte G, Märkl B, Agaimy A. Sclerosing nodular lesions of the gastrointestinal tract containing large numbers of IgG4 plasma cells. Pathology. 2011; 43:31–35.

10. Hiyoshi Y, Oki E, Zaitsu Y, Ando K, Ito S, Saeki H, et al. IgG4-related disease of the ileocecal region mimicking malignancy: a case report. Int J Surg Case Rep. 2014; 5:669–672.

11. Pereira JM, Sirlin CB, Pinto PS, Jeffrey RB, Stella DL, Casola G. Disproportionate fat stranding: a helpful CT sign in patients with acute abdominal pain. Radiographics. 2004; 24:703–715.

12. Rossi M, Virgilio E, Laurino F, Orgera G, Menè P, Pirozzi N, et al. Giant hepatic artery aneurysm associated with immunoglobulin G4-related disease successfully treated using a liquid embolic agent. Korean J Radiol. 2015; 16:953–954.