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autoimmune pancreatitis; lymphoplasmocytic sclerosing pancreatitis

Etiology: 1) type 1 a) associated with IgG4 disease b) may be associated with other IgG4 diseases: - Sjogren's syndrome, sclerosing sialoadenitis - primary sclerosing cholangitis, bile duct strictures - retroperitoneal fibrosis, interstitial nephritis - autoimmune thyroiditis [1] 2) type 2 a) less likely than type 1 to involve elevated serum IgG4 b) associated with chronic pancreatitis & inflammatory bowel disease [1] Epidemiology: - type 1 occurs in older men Pathology: - chronic inflammatory disease - IgG4 bearing lymphocyte/plasma cell inflitrate (type 1) - normal IgG4 positive cell counts in type 2 [1] - may affect biliary tree, salivary glands, retroperitoneum, & lymph nodes in addition to the pancreas Clinical manifestations: - painless jaundice Laboratory: - serum IgG4 is increased (type 1) - serum CA19-9 is normal - also see chronic pancreatitis Special laboratory: - endoscopic ultrasound [1,3] - pancreatic biopsy may be necessary to exclude pancreatic cancer Radiology: - abdominal CT shows diffusely enlarged pancreas (sausage shape) & narrowed main pancreatic duct [1] - follow-up imaging after 3-4 months of glucocorticoid therapy indicated to assess resolution of inflammation Complications: - pancreatic duct stone - pancreatic cancer [5] Differential diagnosis: - pancreatic cancer [3] Management: - glucocorticoids 3-4 month trial [1] - induces remission in almost all patients, (both type 1 & type 2), but relapse is common [1] - patients with relapsed disease generally respond to repeat glucocorticoid treatment [1] - many, but not all, biliary strictures associated with autoimmune pancreatitis respond to glucocorticoids - for patients who fail who fail glucocorticoid therapy, or relapse upon discontinuation, a trial of azathioprine or other immunosuppressive agent is indicated [1] - ERCP with placement of biliary stent in patients who have failed conservative measures

Related

IgG4 disease

General

autoimmune disease chronic pancreatitis

References

  1. Medical Knowledge Self Assessment Program (MKSAP) 16, 17, 18, 19. American College of Physicians, Philadelphia 2012, 2015, 2018, 2021. - Medical Knowledge Self Assessment Program (MKSAP) 19 Board Basics. An Enhancement to MKSAP19. American College of Physicians, Philadelphia 2022
  2. Sugumar A, Chari ST. Diagnosis and treatment of autoimmune pancreatitis. Curr Opin Gastroenterol. 2010 Sep;26(5):513-8 PMID: 20693897
  3. Gardner TB, Levy MJ, Takahashi N, Smyrk TC, Chari ST. Misdiagnosis of autoimmune pancreatitis: a caution to clinicians. Am J Gastroenterol. 2009 Jul;104(7):1620-3. PMID: 19574965
  4. Sah RP, Chari ST, Pannala R, Sugumar A et al Differences in clinical profile and relapse rate of type 1 versus type 2 autoimmune pancreatitis. Gastroenterology. 2010 Jul;139(1):140-8 PMID: 20353791
  5. Hart PA, Kamisawa T, Brugge WR et al Long-term outcomes of autoimmune pancreatitis: a multicentre, international analysis. Gut. 2013 Dec;62(12):1771-6. Epub 2012 Dec 11. PMID: 23232048
  6. Okazaki K, Uchida K. Autoimmune Pancreatitis: The Past, Present, and Future. Pancreas. 2015 Oct;44(7):1006-16. Review. PMID: 26355544
  7. ARUP Consult: Autoimmune Pancreatitis The Physician's Guide to Laboratory Test Selection & Interpretation https://www.arupconsult.com/content/pancreatitis-autoimmune