Autoimmune pancreatitis
Autoimmune pancreatitis (AIP) is a rare autoimmune disease. It represents 2-4% of all chronic pancreatitis. It occurs in two forms differing in geographical distribution, histological picture, and other characteristics:
- Type 1 AIP (lymphoplasmocytic sclerosing pancreatitis) is an IgG4 associated disease located in the pancreas. It rather affects elderly patients.
- Type 2 AIP affects younger patients, the risk of relapse is significantly lower than in type 1 AIP. It is often associated with idiopathic intestinal inflammation.
Both types are characterized by painless jaundice, sudden diabetes mellitus, radiologically detectable pancreatic enlargement, and a history of autoimmune disorders. Both types respond very well to corticosteroid therapy.
The HISORt diagnostic criteria from the Mayo Clinic (2006) for autoimmune pancreatitis are determined in such a way that at least one of the symptom groups must be met for the diagnosis of AIP.
Histopathological finding: at least one of the following must be met:
- lymphoplasmacytic infiltrate with storiform fibrotization and obliterative phlebitis
- lymphoplasmacytic infiltrate with storiform fibrotization and at least 10 IgG4 + plasma cells at the highest magnification
Clinical finding: all criteria must be met
- CT resp. MRI displays diffusely enlarged pancreas with delayed and peripheral gain
- diffusely enlarged and irregular ductus pancreaticus
- the IgG4 level exceeds 1.4 g/l
Therapeutic experiment: all criteria must be met
- unexplained pancreatic disease after excluding other known causes
- elevation of serum IgG4 or histologically confirmed the involvement of another organ with IgG4 + cell infiltration
- remission or significant improvement in pancreatic and/or non-pancreatic disorders after corticosteroid therapy
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References[edit | edit source]
- DITE, P. – NECHUTOVA, H. – UVIROVA, M.. Biomed Pap Med Fac Univ Palacky Olomouc Czech Repub [online]. [cit. 2021-12-9]. <http://biomed.papers.upol.cz/pdfs/bio/2014/01/03.pdf>.
- SUGUMAR, A. – CHARI, S.. Expert Rev Gastroenterol Hepatol. [online]. ©2009. [cit. 2021-12-9]. <https://www.medscape.com/viewarticle/708921_1>.
