Annals of Surgical Oncology

, Volume 15, Issue 4, pp 1147–1154 | Cite as

Elevation of Serum IgG4 in Western Patients With Autoimmune Sclerosing Pancreatocholangitis: A Word of Caution

  • Steven N. Hochwald
  • Alan W. Hemming
  • Peter Draganov
  • Stephen B. Vogel
  • Lisa R. Dixon
  • Stephen R. Grobmyer
Hepatic and Pancreatic Tumors



Autoimmune pancreatocholangitis is characterized by sclerosing inflammation of the biliary tree or pancreatic duct and can mimic pancreaticobiliary malignancy. Serum immunoglobin (Ig) G4 values seem to be helpful in distinguishing autoimmune pancreatocholangitis from pancreatic malignancy in the Japanese population; however, its significance in the Western population has not been well studied.


We report a retrospective analysis of 7 consecutive patients with autoimmune pancreatocholangitis and compare them to 23 patients with pancreatic malignancy. Clinical presentation, diagnostic tests, and preoperative IgG4 levels were reviewed in all patients. Presence of autoimmune pancreatocholangitis or pancreatic malignancy was determined by pathologic analysis in all patients and reviewed by a single pathologist.


In all patients, autoimmune pancreatocholangitis manifested in a similar fashion to pancreatic malignancy. Median IgG4 levels were far lower in pancreatic cancer patients with localized, resectable disease (24 mg/dL), locally advanced disease (24 mg/dL), and metastatic disease (28 mg/dL) as compared with patients with autoimmune pancreatocholangitis (142 mg/dL, P < .05). Only one patient with pancreatic cancer had an IgG4 level that was >100 mg/dL. In contrast, all patients with autoimmune pancreatitis or cholangitis had levels >100 mg/dL. However, in five of these seven patients, IgG4 levels were below the upper limits of normal.


Autoimmune pancreatocholangitis mimics pancreatobiliary malignancy. Serum IgG4 values seem to be helpful in distinguishing autoimmune pancreatocholangitis from malignancy in the Western population. However, absolute values seem to be lower in the United States compared with Japan. The upper limit of normal as reported in laboratories in the United States may not be useful in identifying abnormally high IgG4 values. A new upper limit of normal may need to be defined because IgG subclass determinations are being used more frequently in Western patients with biliary obstruction.


IgG4 Autoimmune pancreatitis Pancreaticobiliary malignancy 


  1. 1.
    Finkelberg DL, Sahani D, Deshpande V, et al. Autoimmune pancreatitis. N Engl J Med 2006;355:2670–6PubMedCrossRefGoogle Scholar
  2. 2.
    Hirano K, Komatsu Y, Yamamoto N, et al. Pancreatic mass lesions associated with raised concentration of IgG4. Am J Gastroenterol 2004;99:2038–40PubMedCrossRefGoogle Scholar
  3. 3.
    Hardacre JM, Iacobuzio-Donahue CA, Sohn TA, et al. Results of pancreatidoduodenectomy for lymphoplasmacytic sclerosing pancreatitis. Ann Surg 2003;237:853–9PubMedCrossRefGoogle Scholar
  4. 4.
    Weber SM, Cubukcu-Dimopulo O, Palesty JA, et al. Lymphoplasmacytic sclerosing pancreatitis: inflammatory mimic of pancreatic carcinoma. J Gastrointest Surg 2003;7:129–39PubMedCrossRefGoogle Scholar
  5. 5.
    Deshpande V, Mino-Kenudson M, Brugge W, et al. Autoimmune pancreatitis. Arch Pathol Lab Med 2005;129:1148–54PubMedGoogle Scholar
  6. 6.
    Hamano H, Kawa S, Horiuchi A, et al. High serum IgG4 concentrations in patients with sclerosing pancreatitis. N Engl J Med 2001;344:732–8PubMedCrossRefGoogle Scholar
  7. 7.
    Schur PH, Rosen F, Norman ME. Immunoglobulin subclasses in normal children. Pediatr Res 1979;13:181–3PubMedCrossRefGoogle Scholar
  8. 8.
    Schur PH. IgG subclasses—a review. Ann Allergy 1987;58:89–96PubMedGoogle Scholar
  9. 9.
    Chen RYM, Adams DB. IgG4 levels in non-Japanese patients with autoimmune sclerosing pancreatitis. N Engl J Med 2002;346:1919PubMedCrossRefGoogle Scholar
  10. 10.
    Kamisawa T, Chen PY, Tu Y, et al. Pancreatic cancer with a high serum IgG4 concentration. World J Gastroenterol 2006;12:6225–8PubMedGoogle Scholar
  11. 11.
    Hughes DB, Grobmyer SR, Brennan MF. Preventing pancreaticoduodenectomy for lymphoplasmacytic sclerosing pancreatitis: cost effectiveness of IgG4. Pancreas 2004;29:167PubMedCrossRefGoogle Scholar
  12. 12.
    Kamisawa T, Tu Y, Nakajima H, et al. Sclerosing cholecystits associated with autoimmune pancreatitis. World J Gastroenterol 2006;12:3736–9PubMedGoogle Scholar
  13. 13.
    Hamano H, Kawa S, Uehara T, et al. Immunoglobulin G4-related lymphoplasmacytic sclerosing cholangitis that mimics infiltrating hilar cholangiocarcinoma: part of a spectrum of autoimmune pancreatitis? Gastroint Endosc 2005;62:152–7CrossRefGoogle Scholar
  14. 14.
    Zen Y, Harada K, Sasaki M, et al. IgG4-related sclerosing cholangitis with and without hepatic inflammatory pseudotumor, and sclerosing pancreatitis-associated sclerosing cholangitis. Am J Surg Pathol 2004;28:1193–203PubMedCrossRefGoogle Scholar

Copyright information

© Society of Surgical Oncology 2008

Authors and Affiliations

  • Steven N. Hochwald
    • 1
  • Alan W. Hemming
    • 2
  • Peter Draganov
    • 3
  • Stephen B. Vogel
    • 1
  • Lisa R. Dixon
    • 4
  • Stephen R. Grobmyer
    • 1
  1. 1.Division of Surgical Oncology, Department of SurgeryUniversity of Florida College of MedicineGainesvilleFlorida
  2. 2.Division of Transplantation and Hepatobiliary Surgery, Department of SurgeryUniversity of Florida College of MedicineGainesvilleFlorida
  3. 3.Division of Gastroenterology, Department of MedicineUniversity of Florida College of MedicineGainesvilleFlorida
  4. 4.Department of Pathology, Immunology and Laboratory MedicineUniversity of Florida College of MedicineGainesvilleFlorida

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