Volume 7, Issue 1 , Pages e3-e4, January 2009
Abdominal Aortitis Associated With Autoimmune Pancreatitis
Article Outline
A 79-year-old man was admitted to our hospital because of body weight loss (7 kg in the past 2 months). Poor appetite, upper abdominal discomfort, and jaundice also were noted. He has had type 2 diabetes mellitus for 10 years and has been using oral hypoglycemic agents. He had not received any surgery previously. On admission, physical examination revealed icteric sclera and yellowish discoloration of his skin. Serum biochemistry tests showed a total bilirubin level of 2.6 mg/dL (normal, 0.2–1.6 mg/dL), direct bilirubin level of 1.5 mg/dL (normal, 0–0.3 mg/dL), immunoglobulin G (IgG) level of 1820 mg/dL (normal, 751–1560 mg/dL), and antinuclear antibody level of 1:160 (normal, <1:80). Other autoantibodies (eg, anti–smooth muscle antibody and antimitochondrial antibody) were negative. The contrast-enhanced abdominal computed tomography (CT) showed a characteristic sausage-like pancreas with a capsule-like low-density rim (Figure A). Intravenous contrast, ionic monomer ioxithalamate (Telebrix 300; Guerbet GmbH Sulzbach, Germany), was injected at the rate of 2.5 mL/s at the initial CT examination. In addition, wall thickening and diffuse enhancement of the lower abdominal aorta were found (Figure B). Endoscopic retrograde cholangiopancreatography depicted diffuse narrowing and irregularity of the main pancreatic duct and segmental narrowing of the distal common bile duct (Figure C). Autoimmune pancreatitis (AIP) with abdominal aortitis, a rare extrapancreatic manifestation of AIP, was diagnosed. The diagnosis of AIP is established according to the revised Japanese criteria.1 We started immunosuppressive therapy with prednisolone. After 3 months of therapy, serum total bilirubin and IgG levels were normal. The follow-up CT showed a marked resolution of abdominal aortitis (Figure D).
AIP frequently is associated with extrapancreatic manifestations such as sclerosing cholangitis, Sjögren's syndrome, ulcerative colitis, psoriasis, sialadenitis, thyroiditis, rheumatoid arthritis, tubulointerstitial nephritis, and retroperitoneal fibrosis.2 We report a case of abdominal aortitis associated with AIP. Histopathologic findings of these extrapancreatic lesions are IgG4+ lymphoplasmacytic infiltration with fibrosis, similar to those of the pancreas. These AIP-associated extrapancreatic lesions are reported to respond well to steroids.3
References
- Clinical diagnostic criteria of autoimmune pancreatitis: revised proposal. J Gastroenterol. 2006;41:626–631
- Systemic extrapancreatic lesions associated with autoimmune pancreatitis. J Gastroenterol. 2007;42(Suppl 18):15–21
- Successful treatment of advanced stage autoimmune pancreatitis-related sclerosing cholangitis. Pancreas. 2006;33:434–437
PII: S1542-3565(08)00892-6
doi:10.1016/j.cgh.2008.08.039
© 2009 AGA Institute. Published by Elsevier Inc. All rights reserved.
Volume 7, Issue 1 , Pages e3-e4, January 2009
