Abstract
Autoimmune pancreatitis is a unique form of chronic pancreatitis characterized by a high serum IgG4 concentration, and complications involving various extrapancreatic lesions. It should be emphasized that autoimmune pancreatitis is easily misdiagnosed as pancreatic cancer. This disease predominantly affects elderly men. The major symptom at onset is obstructive jaundice, while severe abdominal pain is rare. Blood tests have shown abnormal results, which could be attributed mainly to the obstructive jaundice. With regard to autoantibodies, the positive rate for the antinuclear antibody is 40%; however, disease-specific autoantibodies are rarely found. About half of the patients with autoimmune pancreatitis have shown exocrine and endocrine dysfunctions. IgG4 is a sensitive and specific marker for diagnosing autoimmune pancreatitis. In differentiating between pancreatic cancer and autoimmune pancreatitis, IgG4 shows a sensitivity of 90%, a specificity of 98%, and an accuracy of 95%. HLA DRB1*0405-DQB1*0401 alleles are significantly associated with autoimmune pancreatitis, and may present a specific peptide which triggers the autoimmune response. Various imaging findings have shown pancreatic swelling, irregular narrowing of the main pancreatic duct, and stenosis of the lower bile duct. Histology shows a significant presence of lymphocytes, plasma cell infiltration, and fibrosis with abundant IgG4-bearing plasma cell infiltration. After corticosteroid treatment, imaging findings and pancreatic functions usually improve significantly. It is probable that autoimmune pancreatitis is an essentially progressive condition resulting in pancreatic stone formation in a similar way to ordinary chronic pancreatitis.
Similar content being viewed by others
References
H Sarles JC Sarles R Muratore C Guien (1961) ArticleTitleChronic inflammatory sclerosis of the pancreas: an autonomous pancreatic disease? Am J Dig Dis 6 688–98 Occurrence Handle13746542 Occurrence Handle10.1007/BF02232341 Occurrence Handle1:STN:280:CC%2BD28nisFc%3D
S Nakano I Takeda K Kitamura H Watahiki Y Iinuma M Takenaka (1978) ArticleTitleVanishing tumor of the abdomen in patient with Sjögren's syndrome Am J Dig Dis 23 IssueIDSuppl 75S–79S Occurrence Handle10.1007/BF01072494
F Toki T Kozu I Oi T Nakasato M Suzuki F Hanyu (1992) ArticleTitleAn unusual type of chronic pancreatitis showing diffuse irregular narrowing of the entire main pancreatic duct on ERCP: a report of four cases [abstract Endoscopy 24 640
K Kawaguchi M Koike K Tsuruta A Okamoto I Tobata N Fujita (1991) ArticleTitleLymphoplasmacytic sclerosing pancreatitis with cholangitis: a variant of primary sclerosing cholangitis extensively involving the pancreas Hum Pathol 22 387–395 Occurrence Handle2050373 Occurrence Handle10.1016/0046-8177(91)90087-6 Occurrence Handle1:STN:280:By6B28jltlQ%3D
K Yoshida F Toki T Takeuchi S Watanabe K Shiratori N Hayashi (1995) ArticleTitleChronic pancreatitis caused by an autoimmune abnormality. Proposal of the concept of autoimmune pancreatitis Dig Dis Sci 40 1561–8 Occurrence Handle7628283 Occurrence Handle10.1007/BF02285209 Occurrence Handle1:STN:280:ByqA2MvkvFA%3D
T Ito I Nakano S Koyanagi T Miyahara Y Migita K Ogoshi et al. (1997) ArticleTitleAutoimmune pancreatitis as a new clinical entity. Three cases of autoimmune pancreatitis with effective steroid therapy Dig Dis Sci 42 1458–68 Occurrence Handle9246047 Occurrence Handle10.1023/A:1018862626221 Occurrence Handle1:STN:280:ByiA1cfotFA%3D
A Horiuchi S Kawa T Akamatsu Y Aoki K Mukawa N Furuya et al. (1998) ArticleTitleCharacteristic pancreatic duct appearance in autoimmune chronic pancreatitis: a case report and review of the Japanese literature Am J Gastroenterol 93 260–3 Occurrence Handle9468255 Occurrence Handle10.1111/j.1572-0241.1998.00260.x Occurrence Handle1:STN:280:DyaK1c7islOksQ%3D%3D
A Horiuchi S Kawa H Hamano M Hayama H Ota K Kiyosawa (2002) ArticleTitleERCP features in 27 patients with autoimmune pancreatitis Gastrointest Endosc 55 494–499 Occurrence Handle11923760
T Kamisawa N Egawa H Nakajima K Tsuruta A Okamoto N Kamata (2003) ArticleTitleClinical difficulties in the differentiation of autoimmune pancreatitis and pancreatic carcinoma Am J Gastroenterol 98 2694–9 Occurrence Handle14687819 Occurrence Handle10.1111/j.1572-0241.2003.08775.x
A Horiuchi T Kaneko N Yamamura A Nagata T Nakamura T Akamatsu et al. (1996) ArticleTitleAutoimmune chronic pancreatitis simulating pancreatic lymphoma Am J Gastroenterol 91 2607–9 Occurrence Handle8946997 Occurrence Handle1:STN:280:ByiC3c3nsFM%3D
T Wakabayashi Y Motoo Y Kojima H Makino N Sawabu (1998) ArticleTitleChronic pancreatitis with diffuse irregular narrowing of the main pancreatic duct Dig Dis Sci 43 2415–25 Occurrence Handle9824128 Occurrence Handle10.1023/A:1026621913192 Occurrence Handle1:STN:280:DyaK1M%2FjvFOgtg%3D%3D
GW Erkelens FP Vleggaar W Lesterhuis HR van Buuren SD van der Werf (1999) ArticleTitleSclerosing pancreato-cholangitis responsive to steroid therapy Lancet 354 43–4 Occurrence Handle10406367 Occurrence Handle10.1016/S0140-6736(99)00603-0 Occurrence Handle1:STN:280:DyaK1MzjtlSjsg%3D%3D
K Uchida K Okazaki Y Konishi M Ohana H Takakuwa K Hajiro et al. (2000) ArticleTitleClinical analysis of autoimmune-related pancreatitis Am J Gastroenterol 95 2788–94 Occurrence Handle11051349 Occurrence Handle10.1111/j.1572-0241.2000.03187.x Occurrence Handle1:STN:280:DC%2BD3crgt12jtA%3D%3D
K Okazaki K Uchida M Ohana H Nakase S Uose M Inai et al. (2000) ArticleTitleAutoimmune-related pancreatitis is associated with autoantibodies and a Th1/Th2-type cellular immune response Gastroenterology 118 573–81 Occurrence Handle10702209 Occurrence Handle10.1016/S0016-5085(00)70264-2 Occurrence Handle1:CAS:528:DC%2BD3cXit1Smsb0%3D
H Hamano S Kawa A Horiuchi H Unno N Furuya T Akamatsu et al. (2001) ArticleTitleHigh serum IgG4 concentrations in patients with sclerosing pancreatitis N Engl J Med 344 732–8 Occurrence Handle11236777 Occurrence Handle10.1056/NEJM200103083441005 Occurrence Handle1:CAS:528:DC%2BD3MXitFamt7k%3D
K Okazaki T Chiba (2002) ArticleTitleAutoimmune-related pancreatitis Gut 51 1–4 Occurrence Handle12077078 Occurrence Handle10.1136/gut.51.1.1 Occurrence Handle1:STN:280:DC%2BD38zjtl2rtA%3D%3D
H Hamano S Kawa Y Ochi H Unno N Shiba M Wajiki et al. (2002) ArticleTitleHydronephrosis associated with retroperitoneal fibrosis and sclerosing pancreatitis Lancet 359 1403–4 Occurrence Handle11978339 Occurrence Handle10.1016/S0140-6736(02)08359-9
S Kawa H Hamano K Kiyosawa (2006) Pancreatitis NR Rose IR MacKay (Eds) The autoimmune diseases EditionNumber4th ed Academic Press St Louis 779–86
T Nakazawa H Ohara T Yamada H Ando H Sano S Kajino et al. (2001) ArticleTitleAtypical primary sclerosing cholangitis cases associated with unusual pancreatitis Hepatogastroenterology 48 625–30 Occurrence Handle11462890 Occurrence Handle1:STN:280:DC%2BD38%2FitFOntA%3D%3D
T Kuroiwa T Suda T Takahashi H Hirono M Natsui H Motoyama et al. (2002) ArticleTitleBile duct involvement in a case of autoimmune pancreatitis successfully treated with an oral steroid Dig Dis Sci 47 1810–6 Occurrence Handle12184534 Occurrence Handle10.1023/A:1016452813815
T Kamisawa N Funata Y Hayashi K Tsuruta A Okamoto K Amemiya et al. (2003) ArticleTitleClose relationship between autoimmune pancreatitis and multifocal fibrosclerosis Gut 52 683–7 Occurrence Handle12692053 Occurrence Handle10.1136/gut.52.5.683 Occurrence Handle1:STN:280:DC%2BD3s7ntlGqug%3D%3D
H Saegusa M Momose S Kawa H Hamano Y Ochi M Takayama et al. (2003) ArticleTitleHilar and pancreatic gallium-67 accumulation is a characteristic feature of autoimmune pancreatitis Pancreas 27 20–5 Occurrence Handle12826901 Occurrence Handle10.1097/00006676-200307000-00003
K Hirano Y Shiratori Y Komatsu N Yamamoto N Sasahira N Toda et al. (2003) ArticleTitleInvolvement of the biliary system in autoimmune pancreatitis: a follow-up study Clin Gastroenterol Hepatol 1 453–64 Occurrence Handle15017645 Occurrence Handle10.1016/S1542-3565(03)00221-0
A Shinji K Sano H Hamano H Unno M Fukushima N Nakamura et al. (2004) ArticleTitleAutoimmune pancreatitis is closely associated with gastric ulcer presenting with abundant IgG4-bearing plasma cell infiltration Gastrointest Endosc 59 506–11 Occurrence Handle15044886 Occurrence Handle10.1016/S0016-5107(03)02874-8
T Taniguchi M Ko S Seko O Nishida F Inoue H Kobayashi et al. (2004) ArticleTitleInterstitial pneumonia associated with autoimmune pancreatitis Gut 53 770 Occurrence Handle15082601 Occurrence Handle1:STN:280:DC%2BD2c7osF2hsw%3D%3D
S Takeda J Haratake T Kasai C Takaeda E Takazakura (2004) ArticleTitleIgG4-associated idiopathic tubulointerstitial nephritis complicating autoimmune pancreatitis Nephrol Dial Transplant 19 474–6 Occurrence Handle14736977 Occurrence Handle10.1093/ndt/gfg477
K Komatsu H Hamano Y Ochi M Takayama T Muraki K Yoshizawa et al. (2005) ArticleTitleHigh prevalence of hypothyroidism in patients with autoimmune pancreatitis Dig Dis Sci 50 1052–7 Occurrence Handle15986853 Occurrence Handle10.1007/s10620-005-2703-9
I Nishimori A Tamakoshi S Kawa S Tanaka K Takeuchi T Kamisawa et al. (2006) ArticleTitleand the Research Committee on Intractable Pancreatic Diseases, the Ministry of Health and Welfare of Japan. Influence of steroid therapy on the course of diabetes mellitus in patients with autoimmune pancreatitis: findings from a nationwide survey in Japan Pancreas 32 244–8 Occurrence Handle16628078 Occurrence Handle10.1097/01.mpa.0000202950.02988.07 Occurrence Handle1:CAS:528:DC%2BD28Xktlyqtrs%3D
M Takayama H Hamano Y Ochi H Saegusa K Komatsu T Muraki et al. (2004) ArticleTitleRecurrent attacks of autoimmune pancreatitis result in pancreatic stone formation Am J Gastroenterol 99 932–7 Occurrence Handle15128363 Occurrence Handle10.1111/j.1572-0241.2004.04162.x
S Kawa M Ota K Yoshizawa A Horiuchi H Hamano Y Ochi et al. (2002) ArticleTitleHLA DRB10405-DQB10401 haplotype is associated with autoimmune pancreatitis in the Japanese population Gastroenterology 122 1264–9 Occurrence Handle11984513 Occurrence Handle10.1053/gast.2002.33022
K Okazaki S Kawa T Kamisawa T Naruse S Tanaka I Nishimori et al. (2006) ArticleTitleClinical diagnostic criteria of autoimmune pancreatitis: revised proposal J Gastroenterol 41 626–31 Occurrence Handle16932998 Occurrence Handle10.1007/s00535-006-1868-0
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Kawa, S., Hamano, H. Clinical features of autoimmune pancreatitis. J Gastroenterol 42 (Suppl 18), 9–14 (2007). https://doi.org/10.1007/s00535-007-2044-x
Published:
Issue Date:
DOI: https://doi.org/10.1007/s00535-007-2044-x