Autoimmune pancreatitis classification

	Autoimmune pancreatitis Microchapters
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief:

Overview

Autoimmune pancreatitis can be
- Primary (when main pathology is in pancreas) or
- Secondary (when associated with other systemic autoimmune diseases such as IgG4-associated cholangitis, chronic sclerosing sialadenitis, tubulointerstitial nephritis, and ulcerative colitis
Autoimmune pancreatitis may be classified into two types.
- Type 1 AIP
- Type 2 AIP or idiopathic duct-centric pancreatitis

Type 1 AIP meets the HISORt criteria.^[1]^[2]
Type 1 AIP involves pancreas as one part of a systemic IgG4-positive disease.
Type 1 patients have high relapse rates when compared to type 2 patients.^[3]
Type 1 patients are usually older on presentation.
Type 1 patients have higher prevalence of increased IgG4 levels.
Type 1 patients have greater extrapancreatic organ involvement compared to Type 2 AIP patients.

Criteria
(H)	Histology suggestive of autoimmine pancreatitis
(I)	Pancreatic imaging suggestive of autoimmine pancreatitis
(S)	Serology (IgG4 ≥2 times the upper limit of normal)
(O)	Other organ involvement Biliary strictures Parotid/lacrimal gland involvement Mediastinal lymphadenopathy Retroperitoneal fibrosis
(Rt)	Response to steroid treatment

↑ Chari ST, Takahashi N, Levy MJ, Smyrk TC, Clain JE, Pearson RK, Petersen BT, Topazian MA, Vege SS (2009). "A diagnostic strategy to distinguish autoimmune pancreatitis from pancreatic cancer". Clin. Gastroenterol. Hepatol. 7 (10): 1097–103. doi:10.1016/j.cgh.2009.04.020. PMID 19410017.
↑ Chari ST, Smyrk TC, Levy MJ, Topazian MD, Takahashi N, Zhang L, Clain JE, Pearson RK, Petersen BT, Vege SS, Farnell MB (2006). "Diagnosis of autoimmune pancreatitis: the Mayo Clinic experience". Clin. Gastroenterol. Hepatol. 4 (8): 1010–6, quiz 934. doi:10.1016/j.cgh.2006.05.017. PMID 16843735.
↑ Sah RP, Chari ST, Pannala R, Sugumar A, Clain JE, Levy MJ, Pearson RK, Smyrk TC, Petersen BT, Topazian MD, Takahashi N, Farnell MB, Vege SS (2010). "Differences in clinical profile and relapse rate of type 1 versus type 2 autoimmune pancreatitis". Gastroenterology. 139 (1): 140–8, quiz e12–3. doi:10.1053/j.gastro.2010.03.054. PMID 20353791.