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| Autoimmune pancreatitis not otherwise specified (NOS): Clinical features and outcomes of the forgotten type |
| Nicolode Pretis ∗, Filippo Vieceli, Alessandro Brandolese, Lorenzo Brozzi, Antonio Amodio, Luca Frulloni |
Gastroenterology Unit, Pancreas Center, University of Verona, Piazzale L.A. Scuro 10, 37134, Verona, Italy
∗ Corresponding author.
E-mail address: nic_depretis@yahoo.it (N. de Pretis). |
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Abstract Background: Autoimmune pancreatitis (AIP) is a well-recognized fibroinflammatory disease of the pancreas. Despite the significant number of studies published on AIP type 1 and 2, no studies have been focused on AIP type not otherwise specified (NOS) and therefore very little is known about clinical features and long-term outcomes of these patients. The aim of this study was to investigate clinical and radiological features of AIP type NOS-patients.
Methods: Patients classified as AIP type NOS at clinical onset included in our database prospectively maintained since 1995 were evaluated. Epidemiological, clinical data were collected and analyzed.
Results: Forty-six patients were included in the study. The clinical onset was mainly characterized by weight loss, jaundice and acute pancreatitis. Eight patients (17.4%) were reclassified as AIP type 2 during follow-up because of the development of ulcerative colitis. Seven patients (15.2%) experienced relapse after steroid treatment but only one (2.2%) needed immunosuppressive drugs because of recurrent relapses.
Conclusions: AIP type NOS shares clinical features similar to AIP type 2 and a relevant proportion of patients was reclassified as AIP type 2 during follow-up because of the development of ulcerative colitis. The risk of relapse is low but not irrelevant.
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2019, Vol. 18 
