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Presented by Dr. Ashley Cimino-Mathews and prepared by Dr. J. Judd Fite
Clinical History: 20 y/o F with a pancreatic mass.
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Question 1 of 1
1. Question
Clinical History: 20 y/o F with a pancreatic mass
Correct
Answer: C. Autoimmune pancreatitis, type 2
Histology:
This section of pancreas shows regions with normal appearing pancreatic acini and islets, and regions with fibrotic replacement of the parenchyma with residual nodules of ducts and acini. There is marked duct-centric acute inflammation with granulocytic epithelial lesions and abscess formation. There is no evidence of malignancy. There is no evidence of venulitis or prominence of plasma cells. The constellation of features is in keeping with type 2 autoimmune pancreatitis.Discussion:
The constellation of features is in keeping with type 2 autoimmune pancreatitis. Two types of autoimmune pancreatitis have been described. Type 1 (or “lymphoplasmacytic sclerosing pancreatitis”) typically occurs in older male patients and is typically a systemic process related to IgG4 disease, with characteristic findings of obliterative phlebitis and increased numbers of IgG4+ plasma cells. In contrast, type 2 has equal gender distribution and occurs in younger adults than type 1. Type 2 (or “idiopathic duct centric pancreatitis”) is characterized by ductulocentric acute inflammation with granulocyte epithelial lesions and does not have an association with IgG4 disease. The clinical presentations of autoimmune pancreatitis can mimic those of an infiltrating carcinoma. Patients with autoimmune pancreatitis can be treated with steroids.References:
1. Deshpande V, Gupta R, Sainani N, et al. Subclassification of autoimmune pancreatitis: a histologic classification with clinical significance. Am J Surg Pathol. 2011 Jan;35(1):26-35
2. Finkelberg DL, Sahani D, Deshpande V, Brugge WR. Autoimmune pancreatitis. N Engl J Med. 2006 Dec 21;355(25):2670-6.
3. Kamisawa T, Egawa N, Nakajima H . Autoimmune pancreatitis is a systemic autoimmune disease. Am J Gastroenterol. 2003;98(12):2811.
4. Shinagare S, Shinagare AB, Deshpande V. Autoimmune pancreatitis: a guide for the histopathologist. Semin Diagn Pathol. 2012 Nov;29(4):197-204.
5. Stone JH, Zen Y, Deshpande V. IgG4-related disease. N Engl J Med. 2012 Feb;366(6):539-51.Incorrect
Answer: C. Autoimmune pancreatitis, type 2
Histology:
This section of pancreas shows regions with normal appearing pancreatic acini and islets, and regions with fibrotic replacement of the parenchyma with residual nodules of ducts and acini. There is marked duct-centric acute inflammation with granulocytic epithelial lesions and abscess formation. There is no evidence of malignancy. There is no evidence of venulitis or prominence of plasma cells. The constellation of features is in keeping with type 2 autoimmune pancreatitis.Discussion:
The constellation of features is in keeping with type 2 autoimmune pancreatitis. Two types of autoimmune pancreatitis have been described. Type 1 (or “lymphoplasmacytic sclerosing pancreatitis”) typically occurs in older male patients and is typically a systemic process related to IgG4 disease, with characteristic findings of obliterative phlebitis and increased numbers of IgG4+ plasma cells. In contrast, type 2 has equal gender distribution and occurs in younger adults than type 1. Type 2 (or “idiopathic duct centric pancreatitis”) is characterized by ductulocentric acute inflammation with granulocyte epithelial lesions and does not have an association with IgG4 disease. The clinical presentations of autoimmune pancreatitis can mimic those of an infiltrating carcinoma. Patients with autoimmune pancreatitis can be treated with steroids.References:
1. Deshpande V, Gupta R, Sainani N, et al. Subclassification of autoimmune pancreatitis: a histologic classification with clinical significance. Am J Surg Pathol. 2011 Jan;35(1):26-35
2. Finkelberg DL, Sahani D, Deshpande V, Brugge WR. Autoimmune pancreatitis. N Engl J Med. 2006 Dec 21;355(25):2670-6.
3. Kamisawa T, Egawa N, Nakajima H . Autoimmune pancreatitis is a systemic autoimmune disease. Am J Gastroenterol. 2003;98(12):2811.
4. Shinagare S, Shinagare AB, Deshpande V. Autoimmune pancreatitis: a guide for the histopathologist. Semin Diagn Pathol. 2012 Nov;29(4):197-204.
5. Stone JH, Zen Y, Deshpande V. IgG4-related disease. N Engl J Med. 2012 Feb;366(6):539-51.
