Background: Autoimmune pancreatitis is a recently described type of pancreatitis of presumed autoimmune etiology. Autoimmune pancreatitis is often misdiagnosed as pancreatic cancer difficult, since their clinical presentations are often similar. The concept of autoimmune pancreatitis was first published in 1961. Since then, autoimmune pancreatitis has often been treated not as an independent clinical entity but rather as a manifestation of systemic disease. The overall prevalence and incidence of the disease have yet to be determined, but three series have reported the prevalence as between 5 and 6 % of all patients with chronic pancreatitis. Patient vary widely in age, but most are older than 50 years. Patients with autoimmune pancreatitis usually complain of the painless jaundice, mild abdominal pain and weight loss. There is no laboratory hallmark of the disease, even if cholestatic profiles of liver dysfunction with only mild elevation of amylase and lipase levels have been reported.
Conclusions: Proposed diagnostic criteria contains: (1) radiologic imaging, diffuse enlargement of the pancreas and diffusely irregular narrowing of the main pancreatic duct, (2) laboratory data, elevated levels of serum ã-globulin and/or IgG, specially IgG4, or the presence of autoantibodies and (3) histopathologic examination, fibrotic change with dense lymphoplasmacytic infiltration in the pancreas. For correct diagnosis of autoimmune pancreatitis, criterion 1 must be present with criterion 2 and/or 3. Autoimmune pancreatitis is frequently associated with rheumatoid arthritis, Sjogren’s syndrome, inflammatory bowel disease, tubulointersticial nephritis, primary sclerosing cholangitis and idiopathic retroperitoneal fibrosis. Pancreatic biopsy using an endoscopic ultrasound-guided fine needle aspiration biopsy is the most important diagnostic method today. Treatment with corticosteroids leads to the and resolution of pancreatic inflamation, obstruction and pancreatic duct strictures.
Sarles H, Sarles JC, Muratore R. Chronic inflammatory sclerosis of the pancreas: an autonomous pancreatic disease? Am J Dig Dis 1961; 6: 688–98.
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.
Japan Pancreas Society. Diagnostic criteria for autoimmune pancreatitis by the Japan Pancreas Society. J Jpn Pancreas Soc 2002; 17: 585–7.
Finkelbeg DL, Sahani D, Deshpande V, Brugge WR. Autoimmune pancreatitis. N Engl J Med 2006; 355: 2670–6.
Nakazava T, Ohara H, Sano H, Ando T, Imai H, Takada H, et al. Difficulty in diagnosing autoimmune pancreatitis by imaging findings. Gastrointest Endosc 2007; 65: 99–108.
Silverman WB. Autoimmune pancreatitis: more common and important than we thought? Gastrointest Endosc 2007; 65: 109– 10.
Kamisawa T, Egawa N, Nakajima H. Autoimmune pancreatitis is a systemic autoimmune disease. Am J Gastroenterol 2003; 98: 2811–2.
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.
Chari ST. Current Concepts in the treatment of autoimmune pancreatitis. JOP. J Pancreas (Online) 2007; 8: 1–3.
Aparisi L, Farre A, Gomez-Cambronero L. Antibodies to carbonic anhydrase and IgG4 in idiopathic chronic pancreatitis: relevance for diagnosis of autoimmune pancreatitis. Gut 2005; 54: 703–9.
Okazaki K. Autoimmune pancreatitis: etiology, pathogenesis, clinical findings and treatment. The Japanese Experience. JOP. J Pancreas (Online) 2005; 6 Suppl I: 89–96.
Kawa S, Ota M, Yoshizawa K. HLA DRB10405-DQB10401 haplotype is associated with autoimmune pancreatitis in the Japanese population. Gastroenterology 2002; 122: 1264–9.
Kamisawa T, Funata N, Hayashi Y. Close relationship between autoimmune pancreatitis and multifocal fibrosclerosis. Gut 2003; 52: 683–7.
Uchida K, Okazaki K, Nishi T. Experimental immune-mediated pancreatitis in neonatally thymectomized mice immunized with carbonic anhydrase II and lactoferrin. Lab Invest 2002; 82: 411– 24.
Nishimori I, Miyaji E, Morimoto K, Nagao K, Kamada M, Onishi S. Serum antibodies to carbonic anhydrase IV in patients with autoimmune pancreatitis. Gut 2005; 54: 274–81.
Qu WM, Miyazaki T, Terada M, Okada K, Mori S, Kanno H, et al. A novel autoimmune pancreatitis model in MRL mice treated with polyinosinic: polycytidylic acid. Clin Exp Immunol 2002; 129: 27–34.
Okazaki K, Kawa S, Kamisawa T, Naruse S, Tanaka S, Nishimori I, et al. Clinical diagnostic criteria of autoimmune pancreatitis: revised proposal. J Gastroenterol 2006; 41: 626–31.
Kamisawa T, Egawa N, Nakajima H. Extrapancreatic lesions in autoimmune pancreatitis. J Clin Gastroenterol 2005; 39: 904–7.
Irie H, Honda H, Baba S. Autoimmune pancreatitis: CT and MR characteristic. AJR 1998; 170: 1323–7.
Van Hoe L, Gryspeerdt S, Ectors N. Nonalcoholic duct destructive chronic pancreatitis: imaging findings. AJR 1998; 170: 643–7.
Deshpande V, Mino-Kenudsen M, Brugge W. Autoimmune pancreatitis: more than just a pancreatic disease? A contemporary review of its pathology. Arch Pathol Lab Med 2005; 129: 1148– 54.
Pezzilli R, Casadei R, Calculli L, Santini D. Autoimmune pancreatitis. A case mimicking carcinoma. JOP. J Pancreas (Online) 2004; 5: 527–30.
Rudmik L, Trpkov K, Nash C, Kinnear S, Falck V, Dushinski J, et al. Autoimmune pancreatitis associated with renal lesions mimicking metastatic tumours. CMAJ 2006; 75: 367–9.
Sahani DV, Kalva SP, Farrell J. Autoimmune pancreatitis: imaging features. Radiology 2004; 233: 29–35.
Lexy ML. EUS and ERCP in the diagnosis autoimmune pancreatitis. AGA Clinical Symposium Digestive Disease Week, Los Angeles, California, May 21, 2001. Van Hoe L, Gryspeerdt S, Ectors N. Nonalcoholic duct destructive chronic pancreatitis: imaging findings. AJR 1998; 170: 643–7.
Uchida K, Okazaki K, Konishi Y, Ohana M, Takakuwa H, Hajiro K, et al. Clinical analysis of autoimmune-related pancreatitis. Am J Gastroenterol 2000; 95: 2788–94.
Pearson RK, Lognecker DS, Chari ST, Smyrk TC, Okazaki K, Frulloni L, et al. Controversies in clinical pancreatology: autoimmune pancreatitis: does it exist? Pancreas 2003; 27: 1–13.
Kim KP, Kim MH, Song MH, Lee SS, Seo DW, Lee SK. Autoimmune chronic pancreatitis. Am J Gastroenterol 2004; 99: 1605–16.
Zamboni G, Luttges J, Capelli P. Histopathological features of diagnostic and clinical relevance in autoimmune pancreatitis: a study on 53 resection specimens and 9 biopsy specimens. Virchows Arch 2004; 445: 552–63.
Takeda S, Haratake J, Kasai T, Takaeda C, Takazura E. IgG4-associated idiopathic tubulointestinal nephritis complicating autoimmune pancreatitis. Nephrol Dial Transplant 2004; 19: 474–6.
Numata K, Ozawa Y, Kobayashi N. Contrast-enchanced sonography of autoimmune pancreatitis: comparison with pathologic findings. J Ultrasound Med 2004; 23: 199–206.
Sahani DV, Kalva SP, Farrell J. Autoimmune pancreatitis: imaging features. Radiology 2004; 233: 345–52.
Wakabayashi T, Kawaura Y, Satomura Y. Clinical study of chronic pancreatitis with focal irregular narrowing of the main pancreatic duct and mass formation: comparison with chronic pancreatitis showing diffuse irregular narrowing of the main pancreatic duct. Pancreas 2002; 25: 283–9.
Fukumori K, Shakado S, Miyahara T. Atypical manifestation of pancreatitis with autoimmune phenomenon in an adolescent female. Intern Med 2005; 44: 886–91.
Farrell JJ, Garber J, Sahani D, Brugge WR. EUS findings in patients with autoimmune pancreatitis. Gastrointest Endosc 2004; 60: 927–36.
Levy MJ, Reddy RP, Wiersema MJ. EUS-guided trucut biopsy in establishing autoimmune pancreatitis as the cause of obstructive jaudince. Gastrointest Endosc 2005; 61: 467–72.
Mallery JS, Centeno BA, Hahn PF, Chang Y, Warshaw AL, Brugge WR. Pancreatic tissue sampling guided by EUS, CT/US, and surgery: a comparison of sensitivity and specificity. Gastrointest Endosc 2002; 56: 218–24.
Suits J, Frazee R, Erickson RA. Endoscopic ultrasound and fine needle aspiration for the evaluation of pancreatic masses. Arch Surg 1999; 134: 639–42.
Chang KJ, Nguyen P, Erickson RA, Durbin TE, Katz KD. The clinical utility of endoscopic ultrasound-guided fine-needle aspiration in the diagnosis and staging of pancreatic carcinoma. Gastrointest Endosc 1997; 45: 387–93.
Frulloni L, Morana G, Bovo P. Salivary gland involvement in patients with chronic pancreatitis. Pancreas 1999; 19: 33–8.
Hardacre JM, Iacobuzio-Donahue CA, Sohn TA. Results of pancreaticoduodenectomy for lymphoplasmacytic sclerosing pancreatitis. Ann Surg 2003; 237: 853–8.
Ozden I, Dizdaroglu F, Poyanli A. Emre A. Spontaneus regression of a pancreatic head mass and biliary obstruction due to autoimmune pancreatitis. Pancreatology 2005; 5: 300–3.
Kamisawa T, Yoshiike M, Egawa N, Nakajima H, Tsuruta K, Okamoto A. Treating patients with autoimmune pancreatitis: results from a long-term follow-up study. Pancreatology 2003; 3: 234–8.
Kamisawa T, Egawa N, Inokuma S, Tsaruta K, Okamoto A, Kamata N, et al. Pancreatic endocrine and exocrine function and salivary gland function in autoimmune pancreatitis before and after steroid therapy. Pancreas 2003; 27: 235–8.
Toosi MN, Heathcote J. Pancreatic pseudotumor with sclerosing pancreato-cholangitis: Is this a systemic disease? Am J Gastroenterol 2004; 99: 377–82.
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