Autoimmune pancreatitis (AIP) type 1 is commonly associated with simultaneous involvement of extrapancreatic organs. Sclerosing cholangitis, sialadenitis, retroperitoneal fibrosis, Sjögren syndrome, and other extrapancreatic lesions are often observed concurrently with AIP. High levels of immunoglobulin G4 (IgG4) in the blood serum and affected tissues are typical of this diagnostic entity. We describe a case report of a 58-year-old female with findings of AIP (according to Asian criteria), IgG4-positive mastitis, and histologically verified Mikulicz syndrome. The effect of corticoid therapy supported the diagnosis of AIP and simultaneously led to the eradication of recurrent mastitis. To the best of our knowledge, this is the first reported case of concurrent findings of AIP and IgG4 mastitis. Our case report supports the concept of systemic IgG4 syndrome with multisystem involvement. Timely diagnosis and appropriate therapy can be effective in a high percentage of patients.

Department of Internal Medicine and Hepatogastroenterology University Hospital Brno Masaryk University Faculty of Medicine Brno Czech Republic ; Department of Internal Medicine University of Ostrava Faculty of Medicine Ostrava Czech Republic

Zobrazit více v PubMed

Sarles H, Sarles JC, Muratore R, Guien C. Chronic inflammatory sclerosis of the pancreas: an autonomous pancreatic disease? Am J Dig Dis. 1961;6:688–698. PubMed

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;40:1561–1568. PubMed

Park DH, Kim MH, Chari ST. Recent advances in autoimmune pancreatitis. Gut. 2009;58:1680–1689. PubMed

Sugumar A, Smyrk TC, Takahashi N, Levy MJ, Chari ST. Lymphoplasmacytic sclerosing pancreatitis (LPSP) and idiopathic duct centric pancreatis (IDCP) are distinct clinical forms of autoimmune pancreatitis (AIP) Pancreas. 2008;37:497.

Kawaguchi K, Koike M, Tsuruta K, Okamoto A, Tabata I, Fujita N. Lymphoplasmacytic sclerosing pancreatitis with cholangitis: a variant of primary sclerosing cholangitis extensively involving pancreas. Hum Pathol. 1991;22:387–395. PubMed

Kamisawa T, Egawa N, Nakajima H. Autoimmune pancreatitis is a systemic autoimmune disease. Am J Gastroenterol. 2003;98:2811–2812. PubMed

Kamisawa T, Matsukawa M, Ohkawa M. Autoimmune pancreatitis associated with retroperitoneal fibrosis. JOP. 2005;6:260–263. PubMed

Takahashi N, Kawashima A, Fletcher JG, Chari ST. Renal involvement in patients with autoimmune pancreatitis: CT and MR imaging findings. Radiology. 2007;242:791–801. PubMed

Kamisawa T, Nakajima H, Hishima T. Close correlation between chronic sclerosing sialadenitis and immunoglobulin G4. Intern Med J. 2006;36:527–529. PubMed

Kamisawa T, Nakajima H, Egawa N, Funata N, Tsuruta K, Okamoto A. IgG4-related sclerosing disease incorporating sclerosing pancreatitis, cholangitis, sialadenitis and retroperitoneal fibrosis with lymphadenopathy. Pancreatology. 2006;6:132–137. PubMed

Sugumar A, Klöppel G, Chari ST. Autoimmune pancreatitis: pathologic subtypes and their implications for its diagnosis. Am J Gastroenterol. 2009;104:2308–2310. PubMed

Frulloni L, Scattolini C, Falconi M, et al. Autoimmune pancreatitis: differences between the focal and diffuse forms in 87 patients. Am J Gastroenterol. 2009;104:2288–2294. PubMed

Kamisawa T, Funata N, Hayashi Y, et al. A new clinicopathological entity of IgG4-related autoimmune disease. J Gastroenterol. 2003;38:982–984. PubMed

Deshpande V, Chicano S, Finkelberg D, et al. Autoimmune pancreatitis: a systemic immune complex mediated disease. Am J Surg Pathol. 2006;30:1537–1545. PubMed

Deheragoda MG, Church NI, Rodriguez-Justo M, et al. The use of immunoglobulin g4 immunostaining in diagnosing pancreatic and extrapancreatic involvement in autoimmune pancreatitis. Clin Gastroenterol Hepatol. 2007;5:1229–1234. PubMed

Kamisawa T, Okamoto A. Autoimmune pancreatitis: proposal of IgG4-related sclerosing disease. J Gastroenterol. 2006;41:613–625. PubMed PMC

Watson SJ, Jenkins DA, Bellamy CO. Nephropathy in IgG4-related systemic disease. Am J Surg Pathol. 2006;30:1472–1477. PubMed

Hirano K, Kawabe T, Komatsu Y, et al. High-rate pulmonary involvement in autoimmune pancreatitis. Intern Med J. 2006;36:58–61. PubMed

Tsushima K, Tanabe T, Yamamoto H, et al. Pulmonary involvement of autoimmune pancreatitis. Eur J Clin Invest. 2009;39:714–722. PubMed

Otsuki M, Chung JB, Okazaki K, et al. Asian diagnostic criteria for autoimmune pancreatitis: consensus of the Japan-Korea Symposium on Autoimmune Pancreatitis. J Gastroenterol. 2008;43:403–408. PubMed

Chari ST. Diagnosis of autoimmune pancreatitis using its five cardinal features: introducing the Mayo Clinic's HISORt criteria. J Gastroenterol. 2007;42(Suppl 18):39–41. PubMed

Cheuk W, Chan AC, Lam WL, et al. IgG4-related sclerosing mastitis: description of a new member of the IgG4-related sclerosing diseases. Am J Surg Pathol. 2009;33:1058–1064. PubMed

Park do H, Kim MH, Oh HB, et al. Substitution of aspartic acid at position 57 of the DQbeta1 affects relapse of autoimmune pancreatitis. Gastroenterology. 2008;134:440–446. PubMed

Chari ST. Current concepts in the treatment of autoimmune pancreatitis. JOP. 2007;8:1–3. PubMed

Sandanayake NS, Church NI, Chapman MH, et al. Presentation and management of post-treatment relapse in autoimmune pancreatitis/immunoglobulin G4-associated cholangitis. Clin Gastroenterol Hepatol. 2009;7:1089–1096. PubMed

Kamisawa T, Satake K. Therapeutic strategy for autoimmune pancreatitis. Adv Med Sci. 2008;53:145–148. PubMed

Kamisawa T, Okamoto A, Wakabayashi T, Watanabe H, Sawabu N. Appropriate steroid therapy for autoimmune pancreatitis based on long-term outcome. Scand J Gastroenterol. 2008;43:609–613. PubMed