Catching a chameleon: IgG4-related systemic disease

Eu Jin Lim, Prithi S Bhathal, Peter P Tagkalidis and Antony G Speer
Med J Aust 2010; 193 (7): . || doi: 10.5694/j.1326-5377.2010.tb03974.x
Published online: 4 October 2010

IgG4-related systemic disease (IRSD) is a recently described entity with protean manifestations. We describe a patient who developed inflammation and fibrosis in multiple organs over 20 years, sequentially involving his pancreas, bile ducts, gallbladder, submandibular and lacrimal glands, and kidneys. He had an elevated serum IgG4 level. Retrospective analysis of biopsies showed strongly positive tissue immunostaining for IgG4, confirming the diagnosis of IRSD. This case illustrates the natural history of partially treated IRSD and its varied clinical presentations. Early diagnosis and treatment is important, as the condition is highly steroid-responsive.

A 55-year-old man who abstained from drinking alcohol and was not taking any medications initially presented to a hepatobiliary surgeon in 1989 for recurrent abdominal pain and jaundice. On examination, he was icteric, with mild epigastric tenderness, but was afebrile and had no other signs of chronic liver disease. His serum amylase level was mildly elevated, and results of liver function tests (LFTs) showed abnormal levels of albumin (29 g/L; reference range [RR], 36–48 g/L), alkaline phosphatase (284 U/L; RR, 32–91 U/L), γ-glutamyltransferase (98 U/L; RR, < 38 U/L), alanine transaminase (50 U/L; RR, < 34 U/L) and bilirubin (102 μmol/L; RR, < 18 μmol/L).

  • 1 Royal Melbourne Hospital, Melbourne, VIC.
  • 2 Department of Medicine, Royal Melbourne and Western Hospitals, University of Melbourne, Melbourne, VIC.


  • 1. Cheuk W, Yuen HKL, Chu SYY, et al. Lymphadenopathy of IgG4-related sclerosing disease. Am J Surg Pathol 2008; 32: 671-681.
  • 2. Yoshida K, Toki F, Takeuchi T, et al. Chronic pancreatitis caused by an autoimmune abnormality. Proposal of the concept of autoimmune pancreatitis. Dig Dis Sci 1995; 40: 1561-1568.
  • 3. Hamano H, Kawa S, Horiuchi A, et al. High serum IgG4 concentrations in patients with sclerosing pancreatitis. N Engl J Med 2001; 344: 732-738.
  • 4. Tabata M, Kitayama J, Kanemoto H, et al. Autoimmune pancreatitis presenting as a mass in the head of the pancreas: a diagnosis to differentiate from cancer. Am Surg 2003; 69: 363-366.
  • 5. Nakanuma Y, Zen Y. Pathology and immunopathology of immunoglobulin G4-related sclerosing cholangitis: the latest addition to the sclerosing cholangitis family. Hepatol Res 2007; 37 Suppl 3: S478-S486.
  • 6. Ghazale A, Chari ST, Zhang L, et al. Immunoglobulin G4-associated cholangitis: clinical profile and response to therapy. Gastroenterology 2008; 134: 706-715.
  • 7. Tsubota K, Fujita H, Tsuzaka K, Takeuchi T. Mikulicz’s disease and Sjögren’s syndrome. Invest Ophthalmol Vis Sci 2000; 41: 1666-1673.
  • 8. Mehta M, Jakobiec F, Fay A. Idiopathic fibroinflammatory disease of the face, eyelids, and periorbital membrane with immunoglobulin G4-positive plasma cells. Arch Pathol Lab Med 2009; 133: 1251-1255.
  • 9. Watson SJ, Jenkins DA, Bellamy CO. Nephropathy in IgG4-related systemic disease. Am J Surg Pathol 2006; 30: 1472-1477.
  • 10. Khosroshahi A, Stone JR, Pratt DS, et al. Painless jaundice with serial multi-organ dysfunction. Lancet 2009; 373: 1494.


remove_circle_outline Delete Author
add_circle_outline Add Author

Do you have any competing interests to declare? *

I/we agree to assign copyright to the Medical Journal of Australia and agree to the Conditions of publication *
I/we agree to the Terms of use of the Medical Journal of Australia *
Email me when people comment on this article

Online responses are no longer available. Please refer to our instructions for authors page for more information.