Diagnosis, treatment and long-term outcome of autoimmune pancreatitis in Sweden

Vujasinović, Miroslav; Valente, Roberto; Maier, Pia; Beckerath, Victoria von; Haas, Stephan; Arnelo, Urban; Chiaro, Marco Del; Kartalis, Nikolaos; Pozzi-Mucelli, R.; Fernandez-Moro, Carlos; Verbeke, Caroline; Yu, Jingru; W, Ye; Löhr, Matthias

doi:10.1016/j.pan.2018.09.003

Cited by 53 publications

(54 citation statements)

References 38 publications

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“…IgG4-RD is a relatively new clinical entity, and there is no specific definition of its epidemiology because of the multiple knowledge gaps and the fact that many physicians are unfamiliar with the diagnosis [4]. According to our recently published single-centre data, OOI was present in 84% of patients with AIP [5], which is higher when compared with other European studies, which vary from 47% to 61% [6][7][8][9].…”

Section: Introductionmentioning

confidence: 79%

Cardiovascular and Lung Involvement in Patients with Autoimmune Pancreatitis

Nikolic

Brehmer

Panić

et al. 2020

JCM

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Introduction: Immunoglobulin G4-related disease (IgG4-RD) is a systemic immune-mediated disease characterised pathologically by the infiltration of IgG4-bearing plasma cells into the involved organs. Autoimmune pancreatitis (AIP) is a form of chronic pancreatitis with a heavy lymphocytic infiltration and two distinct histopathological subtypes, namely: lymphoplasmacytic sclerosing pancreatitis (AIP type 1) and idiopathic duct-centric pancreatitis (AIP type 2). Lung involvement and aortic involvement have been reported in 12% and 9% of patients with systemic IgG4-RD, respectively. In series including patients with AIP, both lung and aortic involvement were described in 2% of the patients. Most of the epidemiological data come from Japan, and there is a lack of information from Europe, especially the Scandinavian countries. Patients and methods: We performed a single-centre retrospective study on a prospectively collected cohort of patients diagnosed with AIP at the Department for Digestive Diseases at Karolinska University Hospital in Stockholm, Sweden, from 2004 to 2019. Demographic and clinical data were collected from the medical charts. Results: One hundred and thirty-three patients with AIP were analysed. Six patients were excluded because they lacked some of the clinical data relevant to the study. Demographic and clinical features of 127 patients were presented. There were 98 patients with AIP type 1-35 (35.7%) female and 63 (64.3%) male, with a mean age of 55.4 ± 18.2. Among them, 15 (15.3%) patients had lung and/or cardiovascular involvement-11 (11.2%) patients had lung involvement, 10 (10.2%) patients had cardiovascular involvement (six patients had both). Most of them (67.0%) had never smoked. The mean follow-up time of the patients with AIP type 1 was 49 months. Conclusions: Lung and/or cardiovascular involvement were diagnosed in 15 (15.3%) patients in our historical cohort of patients with AIP type 1. Most of the lung involvement was presented in the form of nodular lesions in the lungs, non-specific infiltrates, “ground-glass” appearance with pleura thickening, and effusion. Aortic involvement was a major form of vascular involvement in patients with AIP, as in previous published studies on patients with IgG4-RD.

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Section: Introductionmentioning

confidence: 79%

Cardiovascular and Lung Involvement in Patients with Autoimmune Pancreatitis

Nikolic

Brehmer

Panić

et al. 2020

JCM

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“…A contrast enhanced CT scan may reveal hyperenhancement of the bile duct wall, hypoattenuation of the enlarged pancreas in the arterial phase and isoattenuation or hyperattenuation (compared to the spleen) in the portal venous phase, indicating delayed enhancement (some studies have objectively defined it as an increase of > 15 Hounsfield units from the arterial phase to the portal venous phase). IgG4 serum levels are usually within the normal range with, < 10% of cases presenting mildly elevated levels < 2 × upper limit of normal[ 45 , 89 , 90 ] (Figure 2 ).…”

Section: Idiopathic Duct Centric Chronic Pancreatitis or Type 2 Autoimentioning

confidence: 99%

“…UC is present in 33% to 44% of subjects, either before (47%), simultaneously (32%) or after (21%) the onset of IDCP, and most cases present pancolitis. Crohn’s disease is less frequent, but when present, intestinal involvement seems to be restricted to the colon[ 8 , 89 , 90 ].…”

Section: Idiopathic Duct Centric Chronic Pancreatitis or Type 2 Autoimentioning

confidence: 99%

Steroid-responsive pancreatitides

Peláez-Luna¹,

Soriano-Rios²,

Lira-Treviño³

et al. 2020

WJCC

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Autoimmune pancreatitis has received considerable attention, especially due to the marked effect of corticosteroid therapy on its clinical course. Knowledge, especially regarding type 1 autoimmune pancreatitis, has significantly increased over the last decades, and despite significant differences in pathophysiology and outcomes, both type 1 and 2 autoimmune pancreatitis are still considered different types of the same disease. Some have proposed a different nomenclature reflecting these differences. Although the term steroid-responsive pancreatitides may be interpreted as synonymous to type 1 and 2 autoimmune pancreatitis, these are not the only pancreatic conditions that show a response to steroid therapy. Acute pancreatitis caused by vasculitis and connective tissue diseases and acute pancreatitis secondary to checkpoint inhibitors or programmed cell death receptor antibody-mediated blockage cancer therapy may also benefit from steroid treatment. This review presents current concepts on these disorders, aiming to increase awareness, analyze similarities and differences, and propose a new nomenclature that reflects their specific particularities, clustering them under the term “steroid-responsive pancreatitides”.

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“…Biliary stenting by ERCP [39] and significant focal stenosis of the main pancreatic duct [40] were found as risk factors for formation of pancreatic stones and progression to CP. Exocrine and endocrine insufficiencies were described in a significant number of patients [41], even without detectable changes of advanced pancreatic disease. However, pancreatic enzyme replacement therapy has not been routinely used even in these cases.…”

Section: Treatmentmentioning

confidence: 99%

Autoimmune Pancreatitis: Clinical Presentation and Therapy

Fleiszig¹,

Asencio²

2019

Chronic Autoimmune Epithelitis - Sjogren's Syndrome and Other Autoimmune Diseases of the Exocrine Glands

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Autoimmune pancreatitis is a relatively recently identified entity. The dominant type 1 is the pancreatic manifestation of a systemic IgG4-related fibroinflammatory disease. The type 2 has a clearly different histology, its dominant feature is a granulocytic epithelial lesion, and it is independent of IgG4. While type 1 is rather a disease with male dominance in majors than 50-year-old people, no gender difference is observed in type 2, and the disease is more frequently seen in young people. The more frequent initial clinical manifestation is obstructive jaundice in type 1, while abdominal pain and mild acute pancreatitis in type 2. CT and magnetic resonance images are very similar, IgG4 can be normal even in type 1, and the associated involvement of other organs is frequently posterior to the pancreatic manifestation; thus, the distinction of the two types of AIP can be difficult without histology in the everyday clinical practice. Several cases can be undetermined and qualified as Not Otherwise Specified (NOS). However, all types of AIP respond quickly to steroid treatment with a complete recovery. Late prognosis is good, but up to 50% recurrence has been observed in type 1, and several authors have described progression to chronic pancreatitis.

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Diagnosis, treatment and long-term outcome of autoimmune pancreatitis in Sweden

Cited by 53 publications

References 38 publications

Cardiovascular and Lung Involvement in Patients with Autoimmune Pancreatitis

Cardiovascular and Lung Involvement in Patients with Autoimmune Pancreatitis

Steroid-responsive pancreatitides

Autoimmune Pancreatitis: Clinical Presentation and Therapy

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