Autoimmune Pancreatitis Associated with Various Extrapancreatic ...

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CASE REPORT



Autoimmune Pancreatitis Associated with Various Extrapancreatic Lesions during a Long-term Clinical Course Successfully Treated with Azathioprine and Corticosteroid Maintenance Therapy Itaru Naitoh 1, Takahiro Nakazawa 1, Hirotaka Ohara 1, Hitoshi Sano 1, Tomoaki Ando 1, Kazuki Hayashi 1, Hajime Tanaka 1, Fumihiro Okumura 1, Katsuyuki Miyabe 1, Michihiro Yoshida 1, Satoru Takahashi 2 and Takashi Joh 1

Abstract Autoimmune pancreatitis (AIP) is often associated with systemic extrapancreatic lesions, and the concept of IgG4-related autoimmune disease has been proposed. We report a 62-year-old woman with AIP associated with various extrapancreatic lesions during a long-term clinical course. These extrapancreatic lesions occurred after the withdrawal of steroid therapy. Sclerosing sialadenitis occurred before AIP, whereas autoimmune sensorineural hearing loss, allergic purpura and endocapillary proliferative glomerulonephritis occurred after AIP. However, no relapse was observed for 5 years after the start of maintenance therapy with prednisolone and azathioprine. Key words: autoimmune pancreatitis, autoimmune sensorineural hearing loss, allergic purpura, sclerosing sialadenitis, azathioprine (Inter Med 48: 2003-2007, 2009) (DOI: 10.2169/internalmedicine.48.2695)

Introduction Autoimmune pancreatitis (AIP) is a unique type of chronic pancreatitis whose pathogenesis involves an autoimmune mechanism (1, 2). It is characterized by swelling of the pancreas, irregular narrowing of the main pancreatic duct, and a high serum immunoglobulin (Ig) G4 concentration (3). Immunohistochemically, abundant infiltration of IgG4-positive plasma cells is observed in the pancreas. AIP is often associated with systemic extrapancreatic lesions, and similar immunohistopathological features such as lymphoplasmacytic infiltration and abundant IgG4-positive plasma cells are also observed in some of the extrapancreatic lesions (4-8). Therefore, the concept of IgG4-related autoimmune disease has been proposed (9). Various extrapancreatic lesions have been reported. However, the present

case, which we previously reported as one of the systemic extrapancreatic lesions (10), seems to be the only case of autoimmune sensorineural hearing loss (ASNHL) associated with AIP. AIP and extrapancreatic lesions respond well to steroid therapy. However, some patients with AIP relapse during the clinical course. Steroid is generally used for maintenance therapy and is useful for preventing relapse (11, 12). Recently, some studies have reported the usefulness of azathioprine for the clinical management of relapse in patients with AIP (13, 14). Here, we report a case of AIP in which sclerosing sialadenitis, ASNHL, allergic purpura and endocapillary proliferative glomerulonephritis appeared during a long-term clinical course. These extrapancreatic manifestations did not relapse after the introduction of maintenance therapy with prednisolone (PSL) and azathioprine (AZA).



Department of Gastroenterology and Metabolism, Nagoya City University Graduate School of Medical Sciences, Nagoya and 2Department of Experimental Pathology and Tumor Biology, Nagoya City University Graduate School of Medical Sciences, Nagoya Received for publication July 17, 2009; Accepted for publication August 16, 2009 Correspondence to Dr. Takahiro Nakazawa, [email protected]

2003

Inter Med 48: 2003-2007, 2009

DOI: 10.2169/internalmedicine.48.2695

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Case Report A 62-year-old woman was admitted to our hospital in November 1998 because of abdominal discomfort. She had suffered from right and left chronic sclerosing sialadenitis in 1993 and 1995, and had undergone surgical resection because of a suspected salivary gland tumor. Physical examination revealed no abnormal findings. Laboratory data on admission showed elevated serum levels of alanine aminotransferase, γ-glutamyl transpeptidase and alkaline phosphatase to 66 U/L (normal range 3-35), 541 U/L (5-70) and 451 U/L(100-340), respectively. The serum amylase level was 49 U/L (normal 0-60). The serum γ-globulin, IgG, IgM and IgA levels were 1.33 g/dL(0.7-1.6), 1,685 mg/dL (850-1,650), 255 mg/dL(110-450) and 77 mg/dL (45-190), respectively. Antinuclear antibody (ANA) was positive with a titer of 1 : 640 and a homogeneous pattern. Rheumatoid factor (RF), anti-double stranded DNA antibody, anti-SS-A antibody and anti-SS-B antibody were all negative. Serum levels of CEA and CA19-9 were 36 U/mL (