Usefulness of PET/CT Imaging in Systemic IgG4-related Sclerosing Disease. A Report of Three Cases

  • Vien X Nguyen Department of Gastroenterology and Hepatology, Mayo Clinic. Scottsdale, AZ, USA
  • Giovanni De Petris Department of Pathology, Mayo Clinic. Scottsdale, AZ, USA
  • Ba D Nguyen Department of Radiology, Mayo Clinic. Scottsdale, AZ, USA
Keywords: Autoimmune Diseases, Biological Markers, Immunoglobulin G, Pancreatitis, Plasma Cell Granuloma, Orbital Pseudotumor, Pulmonary, Positron-Emission Tomography, Steroids, Tomography, X-Ray Computed

Abstract

Autoimmune pancreatitis is the pancreatic manifestation of a novel clinicopathological disorder called systemic IgG4-related sclerosing disease. Beside the pancreas, this entity affects other sites (salivary glands, orbit, lung, thyroid, gallbladder, biliary tree system, kidney, abdominal aorta, retroperitoneum, prostate, and lymph node) by infiltration with IgG4-positive plasma cells. Several case reports and small case series have demonstrated the utility of integrated positron emission tomography/computed tomography (PET/CT) in monitoring therapy and documenting relapse and flare-up of autoimmune pancreatitis. However, there are no reports on the usefulness of PET/CT in selecting extrapancreatic sites for tissue sampling. We herein demonstrate the clinical utility of integrated PET/CT in 3 cases of systemic IgG4-related sclerosing disease for targeting extrapancreatic biopsy sites.

Image: Diffuse tracer uptake by the pancreas (crosshairs).

Downloads

Download data is not yet available.

References

Finkelberg DL, Sahani D, Deshpande V, Brugge WR. Autoimmune pancreatitis. N Engl J Med 2006; 355:2670-6. [PMID 17182992]

Sarles H, Sarles JC, Muratore R, Guien C. Chronic inflammatory sclerosis of the pancreas--an autonomous pancreatic disease? Am J Dig Dis 1961; 6:688-98. [PMID 13746542]

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; 40:1561-8. [PMID 7628283]

Hamano H, Kawa S, Ochi Y, Unno H, Shiba N, Wajiki M, et al. Hydronephrosis associated with retroperitoneal fibrosis and sclerosing pancreatitis. Lancet 2002; 359:1403-4. [PMID 11978339]

Kamisawa T, Funata N, Hayashi Y, Eishi Y, Koike M, Tsuruta K, et al. A new clinicopathological entity of IgG4-related autoimmune disease. J Gastroenterol 2003; 38:982-4. [PMID 14614606]

Nakajo M, Jinnouchi S, Noguchi M, Uozumi K, Tanabe H, Tateno R, et al. FDG PET and PET/CT monitoring of autoimmune pancreatitis associated with extrapancreatic autoimmune disease. Clin Nucl Med 2007; 32:282-5. [PMID 17413574]

Kawamura E, Habu D, Higashiyama S, Tsushima H, Shimonishi Y, Nakayama Y, et al. A case of sclerosing cholangitis with autoimmune pancreatitis evaluated by FDG-PET. Ann Nucl Med 2007; 21:223-8. [PMID 17581721]

Otsuka H, Morita N, Yamashita K, Nishitani H. FDG-PET/CT findings of autoimmune pancreatitis associated with idiopathic retroperitoneal fibrosis. Ann Nucl Med 2007; 21:593-6. [PMID 18092136]

Nakajo M, Jinnouchi S, Fukukura Y, Tanabe H, Tateno R, Nakajo M. The efficacy of whole-body FDG-PET or PET/CT for autoimmune pancreatitis and associated extrapancreatic autoimmune lesions. Eur J Nucl Med Mol Imaging 2007; 34:2088-95. [PMID 17713765]

Matsubayashi H, Furukawa H, Maeda A, Matsunaga K, Kanemoto H, Uesaka K, et al. Usefulness of positron emission tomography in the evaluation of distribution and activity of systemic lesions associated with autoimmune pancreatitis. Pancreatology 2009; 9:694-9. [PMID 19684434]

Lee TY, Kim MH, Park do H, Seo DW, Lee SK, Kim JS, et al. Utility of 18F-FDG PET/CT for differentiation of autoimmune pancreatitis with atypical pancreatic imaging findings from pancreatic cancer. AJR Am J Roentgenol 2009; 193:343-8. [PMID 19620430]

Ozaki Y, Oguchi K, Hamano H, Arakura N, Muraki T, Kiyosawa K, et al. Differentiation of autoimmune pancreatitis from suspected pancreatic cancer by fluorine-18 fluorodeoxyglucose positron emission tomography. J Gastroenterol 2008; 43:144-51. [PMID 18306988]

Sato M, Okumura T, Shioyama Y, Imura J. Extrapancreatic F-18 FDG accumulation in autoimmune pancreatitis. Ann Nucl Med 2008; 22:215-9. [PMID 18498037]

Shigekawa M, Yamao K, Sawaki A, Hara K, Takagi T, Bhatia V, et al. Is (18)F-fluorodeoxyglucose positron emission tomography meaningful for estimating the efficacy of corticosteroid therapy in patients with autoimmune pancreatitis? J Hepatobiliary Pancreat Sci 2010; 17:269-74. [PMID 19727541]

Kamisawa T, Takum K, Anjiki H, Egawa N, Kurata M, Honda G, et al. FDG-PET/CT findings of autoimmune pancreatitis. Hepatogastroenterology 2010; 57:447-50. [PMID 20698206]

Kamisawa T, Okamoto A. IgG4-related sclerosing disease. World J Gastroenterol 2008; 14:3948-55. [PMID 18609677]

Chari ST, Smyrk TC, Levy MJ, Topazian MD, Takahashi N, Zhang L, et al. Diagnosis of autoimmune pancreatitis: the Mayo Clinic experience. Clin Gastroenterol Hepatol 2006; 4:1010-6. [PMID 16843735]

Kamisawa T. Diagnostic criteria for autoimmune pancreatitis. J Clin Gastroenterol 2008; 42:404-7. [PMID 18277897]

Sugumar A, Chari ST. Diagnosis and treatment of autoimmune pancreatitis. Curr Opin Gastroenterol 2010; 26:513-8. [PMID 20693897]

Abraham SC, Wilentz RE, Yeo CJ, Sohn TA, Cameron JL, Boitnott JK, Hruban RH. Pancreaticoduodenectomy (Whipple resections) in patients without malignancy: are they all 'chronic pancreatitis'? Am J Surg Pathol 2003; 27:110-20. [PMID 12502933]

Gardner TB, Levy MJ, Takahashi N, Smyrk TC, Chari ST. Misdiagnosis of autoimmune pancreatitis: a caution to clinicians. Am J Gastroenterol 2009; 104:1620-3. [PMID 19574965]

Hamano H, Kawa S, Horiuchi A, Unno H, Furuya N, Akamatsu T, et al. High serum IgG4 concentrations in patients with sclerosing pancreatitis. N Engl J Med 2001; 344:732-8. [PMID 11236777]

Kamisawa T, Okamoto A, Funata N. Clinicopathological features of autoimmune pancreatitis in relation to elevation of serum IgG4. Pancreas 2005; 31:28-31. [PMID 15968244]

Kwon S, Kim MH, Choi EK. The diagnostic criteria for autoimmune chronic pancreatitis: it is time to make a consensus. Pancreas 2007; 34:279-86. [PMID 17414049]

Ghazale A, Chari ST, Smyrk TC, Levy MJ, Topazian MD, Takahashi N, et al. Value of serum IgG4 in the diagnosis of autoimmune pancreatitis and in distinguishing it from pancreatic cancer. Am J Gastroenterol 2007; 102:1646-53. [PMID 17555461]

Morselli-Labate AM, Pezzilli R. Usefulness of serum IgG4 in the diagnosis and follow up of autoimmune pancreatitis: A systematic literature review and meta-analysis. J Gastroenterol Hepatol 2009; 24:15-36. [PMID 19067780]

Zen Y, Fujii T, Harada K, Kawano M, Yamada K, Takahira M, et al. Th2 and regulatory immune reactions are increased in immunoglobin G4-related sclerosing pancreatitis and cholangitis. Hepatology 2007; 45:1538-46. [PMID 17518371]

Kamisawa T, Anjiki H, Egawa N, Kubota N. Allergic manifestations in autoimmune pancreatitis. Eur J Gastroenterol Hepatol 2009; 21:1136-9. [PMID 19757521]

Taniguchi T, Ko M, Seko S, Nishida O, Inoue F, Kobayashi H, et al. Interstitial pneumonia associated with autoimmune pancreatitis. Gut 2004; 53:770-1. [PMID 15082601]

Zen Y, Kitagawa S, Minato H, Kurumaya H, Katayanagi K, Masuda S, et al. IgG4-positive plasma cells in inflammatory pseudotumor (plasma cell granuloma) of the lung. Hum Pathol 2005; 36:710-7. [PMID 16084938]

Tsushima K, Tanabe T, Yamamoto H, Koizumi T, Kawa S, Hamano H, et al. Pulmonary involvement of autoimmune pancreatitis. Eur J Clin Invest 2009; 39:714-22. [PMID 19496802]

Fujinaga Y, Kadoya M, Kawa S, Hamano H, Ueda K, Momose M, et al. Characteristic findings in images of extra-pancreatic lesions associated with autoimmune pancreatitis. Eur J Radiol 2010; 76:228-38. [PMID 19581062]

Ghazale A, Chari ST, Zhang L, Smyrk TC, Takahashi N, Levy MJ, et al. Immunoglobulin G4-associated cholangitis: clinical profile and response to therapy. Gastroenterology 2008; 134:706-15. [PMID 18222442]

Sandanayake NS, Church NI, Chapman MH, Johnson GJ, Dhar DK, Amin Z, et al. Presentation and management of post-treatment relapse in autoimmune pancreatitis/immunoglobulin G4-associated cholangitis. Clin Gastroenterol Hepatol 2009; 7:1089-96. [PMID 19345283]

Topazian M, Witzig TE, Smyrk TC, Pulido JS, Levy MJ, Kamath PS, et al. Rituximab therapy for refractory biliary strictures in immunoglobulin G4-associated cholangitis. Clin Gastroenterol Hepatol 2008; 6:364-6. [PMID 18328441]

Khosroshahi A, Bloch DB, Deshpande V, Stone JH. Rituximab therapy leads to rapid decline of serum IgG4 levels and prompt clinical improvement in IgG4-related systemic disease. Arthritis Rheum 2010; 62:1755-62. [PMID 20191576]

Reeder CB, Reece DE, Kukreti V, Chen C, Trudel S, Hentz J, et al. Cyclophosphamide, bortezomib and dexamethasone induction for newly diagnosed multiple myeloma: high response rates in a phase II clinical trial. Leukemia 2009; 23:1337-41. [PMID 19225538]

Manz RA, Hauser AE, Hiepe F, Radbruch A. Maintenance of serum antibody levels. Annu Rev Immunol 2005; 23:367-86. [PMID 15771575]

Neubert K, Meister S, Moser K, Weisel F, Maseda D, Amann K, et al. The proteasome inhibitor bortezomib depletes plasma cells and protects mice with lupus-like disease from nephritis. Nat Med 2008; 14:748-55. [PMID 18542049]

Diffuse tracer uptake by the pancreas (crosshairs)
Published
2011-05-06
How to Cite
NguyenV., De PetrisG., & NguyenB. (2011). Usefulness of PET/CT Imaging in Systemic IgG4-related Sclerosing Disease. A Report of Three Cases. JOP. Journal of the Pancreas, 12(3), 297-305. https://doi.org/10.6092/1590-8577/3302
Section
MULTIMEDIA ARTICLE - Clinical Imaging

Most read articles by the same author(s)