Autoimmune pancreatitis not otherwise specified (NOS): Clinical features and outcomes of the forgotten type.

Published on Jun 11, 2019in Hepatobiliary & Pancreatic Diseases International2.428
· DOI :10.1016/J.HBPD.2019.05.010
Nicolò de Pretis8
Estimated H-index: 8
(University of Verona),
Filippo Vieceli2
Estimated H-index: 2
(University of Verona)
+ 3 AuthorsLuca Frulloni58
Estimated H-index: 58
(University of Verona)
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.
📖 Papers frequently viewed together
4 Authors (Sebastian Rasch, ..., Hana Algül)
12 Citations
15 Citations
10 Citations
#1Matteo Tacelli (University of Palermo)H-Index: 5
#2Ciro Celsa (University of Palermo)H-Index: 10
Last. Calogero Cammà (University of Palermo)H-Index: 76
view all 9 authors...
Background & Aims Risk for relapse after induction of remission with steroid therapy has been studied extensively in patients with autoimmune pancreatitis (AIP), but findings have been equivocal. We performed a systematic review and meta-analysis to estimate the relapse rate of AIP after initial remission after steroid treatment and to identify factors associated with relapse. Methods Three reviewers searched MEDLINE, SCOPUS, and EMBASE until July 2018 to identify studies on rate of relapse of A...
9 CitationsSource
#1Hyun Woo Lee (SCH: Soonchunhyang University)H-Index: 4
#2Sung-Hoon Moon (Sacred Heart Hospital)H-Index: 8
Last. Sung Koo Lee (UOU: University of Ulsan)H-Index: 69
view all 11 authors...
Background Type 1 autoimmune pancreatitis (AIP), as a pancreatic manifestation of IgG4-related disease, shows a favorable prognosis in the short term. However, disease relapse is common in long-term follow-up, despite a successful initial treatment response. This study aimed to identify the predictors of relapse and long-term outcomes in patients with type 1 AIP.
23 CitationsSource
#1Masaki Miyazawa (Kanazawa University)H-Index: 6
#2Hajime Takatori (Kanazawa University)H-Index: 13
Last. Shuichi Kaneko (Kanazawa University)H-Index: 104
view all 19 authors...
Background and aim Relapse and diabetes mellitus (DM) are major problems for the prognosis of autoimmune pancreatitis (AIP). We examined the prognosis of type 1 AIP after corticosteroid therapy (CST)-induced remission in terms of relapse and DM. Methods The study enrolled 82 patients diagnosed with type 1 AIP who achieved remission with CST. We retrospectively evaluated the relapse rate in terms of the administration period of CST, clinical factors associated with relapse, and the temporal chang...
15 CitationsSource
#1Atsushi Masamune (Tohoku University)H-Index: 67
#2Isao NishimoriH-Index: 55
Last. Tooru Shimosegawa (Tohoku University)H-Index: 95
view all 29 authors...
Objective Corticosteroid has been established as the standard therapy for autoimmune pancreatitis (AIP), but the requirement for maintenance corticosteroid therapy is controversial. We conducted a randomised controlled trial to clarify the efficacy of maintenance corticosteroid therapy in patients with AIP. Design We conducted a multicentre, tertiary setting, randomised controlled trial. After the induction of remission with the initial oral prednisolone (PSL) treatment, maintenance therapy with...
77 CitationsSource
#1Kensuke Kubota (YCU: Yokohama City University)H-Index: 32
#2Terumi KamisawaH-Index: 77
Last. Tsutomu Chiba (Kyoto University)H-Index: 105
view all 26 authors...
Background The effect of maintenance steroid treatment (MST) in reducing the risk of relapse in patients with autoimmune pancreatitis (AIP) remains under debate. The aim of this study was to validate the effect of MST on AIP administered in accordance with the 2010 Japanese consensus guidelines.
27 CitationsSource
#1Kazuichi Okazaki (Kansai Medical University)H-Index: 85
#2Suresh T. Chari (Mayo Clinic)H-Index: 103
Last. Tooru Shimosegawa (Tohoku University)H-Index: 95
view all 11 authors...
Abstract Background and aims The International Consensus Diagnostic Criteria (ICDC) for AIP has proposed two distinctive type of AIP, type 1 and type 2, and enabled us first to differentiate two types of AIP each other. By initial steroid treatment for induction of remission, remission can be successfully induced in almost all subjects with type 1 and type 2 AIP. As relapse rate in type 1 AIP is significantly higher than in type 2 AIP, there has been ongoing debate on how to treat effectively re...
71 CitationsSource
#1Phil A. Hart (Mayo Clinic)H-Index: 29
#2Michael J. Levy (Mayo Clinic)H-Index: 107
Last. Suresh T. Chari (Mayo Clinic)H-Index: 103
view all 13 authors...
Objective Idiopathic duct-centric chronic pancreatitis (IDCP), also known as type 2 autoimmune pancreatitis (AIP), is an uncommon subtype of AIP. International Consensus Diagnostic Criteria for IDCP propose that the diagnosis requires pancreatic histology and/or concurrent IBD. We examined our experience with IDCP (type 2 AIP) to assess the appropriateness of these criteria, and identify unique characteristics in patients presenting with acute pancreatitis. Design We reviewed the Mayo Clinic AIP...
34 CitationsSource
#1Kenji NotoharaH-Index: 41
#1Kenji Notohara (Kansai Medical University)H-Index: 6
Last. Tooru ShimosegawaH-Index: 95
view all 11 authors...
The aim of the study was to clarify clinicopathological features of type 2 autoimmune pancreatitis (AIP) in Japan; a multicenter survey was carried out.The first screening collected patients with pancreatitis whose pancreatic tissue samples were available and who fulfilled at least 1 of the following 3 criteria as possible type 2 AIP: (1) histological presence of granulocytic epithelial lesion, (2) age of 50 years or younger, and (3) association of ulcerative colitis, Sjogren syndrome, and/or pr...
18 CitationsSource
#1Phil A. Hart (Mayo Clinic)H-Index: 29
#2Terumi Kamisawa (Tokyo Metropolitan Komagome Hospital)H-Index: 77
Last. Suresh T. Chari (Mayo Clinic)H-Index: 103
view all 25 authors...
Objective Autoimmune pancreatitis (AIP) is a treatable form of chronic pancreatitis that has been increasingly recognised over the last decade. We set out to better understand the current burden of AIP at several academic institutions diagnosed using the International Consensus Diagnostic Criteria, and to describe long-term outcomes, including organs involved, treatments, relapse frequency and long-term sequelae. Design 23 institutions from 10 different countries participated in this multination...
317 CitationsSource
#1Terumi Kamisawa (Tokyo Metropolitan Komagome Hospital)H-Index: 77
#2Suresh T. Chari (Mayo Clinic)H-Index: 103
Last. Tooru Shimosegawa (Tohoku University)H-Index: 95
view all 6 authors...
Autoimmune pancreatitis (AIP) is a form of chronic pancreatitis characterised clinically by frequent presentation with obstructive jaundice, histologically by a lymphoplasmacytic infiltrate with fibrosis, and therapeutically by a dramatic response to steroids. When so defined, AIP can be sub-classified into two subtypes, 1 and 2. Recent international consensus diagnostic criteria for AIP have been developed for diagnosis of both forms of AIP. Type 1 AIP is the pancreatic manifestation of a multi...
115 CitationsSource
Cited By2
IgG4-related disease is a recently defined clinical entity that can manifest itself in any organ. The most common gastrointestinal manifestations are diseases of the pancreas (autoimmune pancreatitis type 1) and biliary tree (IgG4-associated cholangitis); involvement of liver parenchyma is uncommon and the affection of tubular organs is very rare. IgG4-related pancreatitis and cholangitis can mimic malignancies in their clinical presentation. Diagnosis is often difficult and requires careful eva...
#1Annarosa Floreani (UNIPD: University of Padua)H-Index: 59
#2Kazuichi Okazaki (Kansai Medical University)H-Index: 85
Last. M. Eric Gershwin (UC Davis: University of California, Davis)H-Index: 139
view all 4 authors...
IgG4-related disease (IgG4-RD) represents an immune-mediated fibroinflammatory condition with peculiar histopathologic changes that can affect various organs. In 2012 its unified nomenclature was published, which allows to abandon other synonymous names. Up to now, only little is known about its epidemiology around the world. However, although it is generally considered a rare condition, the number of patients with IgG4-RD is increasing enormously. Likewise, the annual number of publications on ...
#1Sönke Detlefsen (University of Southern Denmark)H-Index: 21
#2Søren Schou Olesen (AAU: Aalborg University)H-Index: 28
Abstract Autoimmune pancreatitis (AIP) is a distinct form of chronic pancreatitis that has been increasingly recognised over the last decades and shows a good response to corticosteroid treatment. Two different forms of AIP have been characterized. Type 1 AIP is the pancreatic manifestation of IgG4-related disease and often affects multiple organ systems. In contrast, type 2 AIP is confined to the pancreas and involvement of extra-pancreatic organs has previously only very rarely been reported, ...
PURPOSE OF REVIEW Type 2 autoimmune pancreatitis (AIP) is a rare inflammatory disease of the pancreas. Very few data have been published on this particular subtype, which differs from the 'classical' IgG4-related type 1 AIP in terms of pathological features, epidemiology and risk of relapse. The aim of the current review is to summarize the available literature, suggesting a diagnostic and therapeutic approach to this disease. RECENT FINDINGS Based on the International Consensus Diagnostic Crite...