Correspondence

IgG4 Levels in Non-Japanese Patients with Autoimmune Sclerosing Pancreatitis

N Engl J Med 2002; 346:1919June 13, 2002

Article

To the Editor:

Autoimmune sclerosing pancreatitis has been reported to be associated with elevated IgG4 levels in Japanese patients.1 It is unknown whether similar elevation in IgG4 levels is present in non-Japanese patients with this condition. Two patients have recently been identified in our institute with autoimmune sclerosing pancreatitis, and their IgG4 levels have been measured.

Patient 1 was a 49-year-old white man presenting with a nine-month history of intermittent attacks of epigastric pain, obstructive jaundice, and fever. Imaging studies showed a diffusely enlarged pancreas (on computed tomography), diffuse hypoechoic enlargement of the pancreas (on endoscopic ultrasonography), a long segment of extrahepatic biliary stricture, and an irregular pancreatic duct (on endoscopic retrograde cholangiopancreatography). The patient eventually underwent a Whipple procedure because of concern about cancer. The final histologic examination of the resected specimen showed autoimmune sclerosing pancreatitis with lymphoplasmacytic infiltration and fibrosis.2

Patient 2 was a 56-year-old black woman with two previous episodes of pancreatitis, persistent abdominal pain, and weight loss. Endoscopic retrograde cholangiopancreatography showed stricture at the junction of the body and the tail of the pancreas with dilatation of the pancreatic duct at the tail. The possibility of a neoplastic process was raised, and the patient underwent distal pancreatectomy. Histologic examination showed the presence of autoimmune sclerosing pancreatitis. After diagnosis, total IgG and IgG4 levels were obtained in these patients (seven months and one month after surgery in Patient 1 and Patient 2, respectively). The IgG4 levels were found to be elevated in both patients (159 mg per deciliter in Patient 1 and 182 mg per deciliter in Patient 2; range, 7 to 89 mg per deciliter), although total IgG levels were normal (1180 mg per deciliter in Patient 1 and 1300 mg per deciliter in Patient 2; range, 694 to 1618 mg per deciliter). Other autoantibodies — antinuclear antibodies, anti–double-stranded DNA antibodies, rheumatoid factor, antithyroglobulin antibodies, and antimicrosomal antibodies — were not present.

Although IgG4 levels have been shown to be elevated in Japanese patients with autoimmune sclerosing pancreatitis, it is unclear whether they are elevated in patients of other races or ethnic groups. We found that IgG4 was elevated in both a white man and a black woman, suggesting that elevation of IgG4 might be useful for the identification of patients with autoimmune sclerosing pancreatitis, which is usually difficult to diagnose preoperatively.

Robert Y.M. Chen, M.D.
David B. Adams, M.D.
Medical University of South Carolina, Charleston, SC 29425
chenrobe@musc.edu

2 References

1. 1

   Hamano H, Kawa S, Horiuchi A, et al. High serum IgG concentrations in patients with sclerosing pancreatitis. N Engl J Med 2001;344:732-738
   Full Text | Web of Science | Medline

2. 2

   Yoshida K, Toki F, Takeuchi T, Watanabe S, Shiratori K, Hayashi N. Chronic pancreatitis caused by an autoimmune abnormality: proposal of the concept of autoimmune pancreatitis. Dig Dis Sci 1995;40:1561-1568
   CrossRef | Web of Science | Medline

Citing Articles (14)

Citing Articles

1. 1

   Steve M.M. De Castro, Lindsey C.F. De Nes, C. Yung Nio, Daan C. Velseboer, Fiebo J.W. Ten Kate, Olivier R.C. Busch, Thomas M. Van Gulik, Dirk Jan Gouma. (2010) Incidence and characteristics of chronic and lymphoplasmacytic sclerosing pancreatitis in patients scheduled to undergo a pancreatoduodenectomy. HPB 12:1, 15-21
   CrossRef

2. 2

   Erin Barth, Thomas J Savides. (2009) Autoimmune pancreatitis. Expert Review of Clinical Immunology 5:6, 801-811
   CrossRef

3. 3

   Wouter Van Moerkercke, Marc Verhamme, Gert Meeus, Raymond Oyen, Werner Van Steenbergen. (2009) A Case of IgG4-Related Sclerosing Disease With Retroperitoneal Fibrosis, Autoimmune Pancreatitis and Bilateral Focal Nephritis. Pancreas 38:7, 825-832
   CrossRef

4. 4

   Antonio M Morselli-Labate, Raffaele Pezzilli. (2009) Usefulness of serum IgG4 in the diagnosis and follow up of autoimmune pancreatitis: A systematic literature review and meta-analysis. Journal of Gastroenterology and Hepatology 24:1, 15-36
   CrossRef

5. 5

   Steven N. Hochwald, Alan W. Hemming, Peter Draganov, Stephen B. Vogel, Lisa R. Dixon, Stephen R. Grobmyer. (2008) Elevation of Serum IgG4 in Western Patients With Autoimmune Sclerosing Pancreatocholangitis: A Word of Caution. Annals of Surgical Oncology 15:4, 1147-1154
   CrossRef

6. 6

   Petr Dite, Ivo Novotny, Jan Trna, Arona Sevcikova. (2008) Autoimmune pancreatitis. Best Practice & Research Clinical Gastroenterology 22:1, 131-143
   CrossRef

7. 7

   Masayuki Matsuda, Hideaki Hamano, Takuhiro Yoshida, Takahisa Gono, Takeshi Uehara, Shigeyuki Kawa, Shu-ichi Ikeda. (2007) Seronegative Sjögren syndrome with asymptomatic autoimmune sclerosing pancreatitis. Clinical Rheumatology 26:1, 117-119
   CrossRef

8. 8

   Vikram Deshpande, Sonia Chiocca, Dmitry Finkelberg, Martin K. Selig, Mari Mino-Kenudson, William R. Brugge, Robert B. Colvin, Gregory Y. Lauwers. (2006) Autoimmune Pancreatitis: A Systemic Immune Complex Mediated Disease. The American Journal of Surgical Pathology 30:12, 1537-1545
   CrossRef

9. 9

   Vikram Deshpande, Mari Mino-Kenudson, William R Brugge, Martha B Pitman, Carlos Fernandez-del Castillo, Andrew L Warshaw, Gregory Y Lauwers. (2005) Endoscopic Ultrasound Guided Fine Needle Aspiration Biopsy of Autoimmune Pancreatitis. The American Journal of Surgical Pathology 29:11, 1464-1471
   CrossRef

10. 10

    Terumi Kamisawa, Masami Yoshiike, Naoto Egawa, Hitoshi Nakajima, Kouji Tsuruta, Atsutake Okamoto. (2005) Treating Patients with Autoimmune Pancreatitis: Results from a Long-Term Follow-Up Study. Pancreatology 5:2-3, 234-240
    CrossRef

11. 11

    Eugene P DiMagno. (2003) Autoimmune chronic pancreatitis: a plea for simplification and consistency. Clinical Gastroenterology and Hepatology 1:6, 421-422
    CrossRef

12. 12

    Matthew J. DiMagno, Eugene P. DiMagno. (2003) Chronic pancreatitis. Current Opinion in Gastroenterology 19:5, 451-457
    CrossRef

13. 13

    Randall K. Pearson, Daniel S. Longnecker, Suresh T. Chari, Thomas C. Smyrk, Kazuichi Okazaki, Luca Frulloni, Giorgio Cavallini. (2003) Controversies in Clinical Pancreatology. Pancreas 27:1, 1-13
    CrossRef

14. 14

    Jeffrey M. Hardacre, Christine A. Iacobuzio-Donahue, Taylor A. Sohn, Susan C. Abraham, Charles J. Yeo, Keith D. Lillemoe, Michael A. Choti, Kurtis A. Campbell, Richard D. Schulick, Ralph H. Hruban, John L. Cameron, Steven D. Leach. (2003) Results of Pancreaticoduodenectomy for Lymphoplasmacytic Sclerosing Pancreatitis. Annals of Surgery 237:6, 853-859
    CrossRef