Autoimmune Pancreatitis and Ulcerative Rectocolitis in an Adolescent

Background and Objectives: Inflammatory bowel disease (IBD) is a chronic condition and mainly affects the intestines, however, the involvement of the other organs of the gastrointestinal tract (upper part, pancreas, and liver) have been observed. The coexistence of IBD with pancreatic pathology is rare, however, it has been diagnosed more frequently during recent years in the pediatric population. This article reviews the current literature on the most common pancreatic diseases associated with IBD in the pediatric population and their relationship with IBD activity and treatment. Materials and Methods: We performed a systematic review of data from published studies on pancreatic disorders, also reported as extraintestinal manifestations (EIMs), among children with IBD. We searched PubMed and Web of Science to identify eligible studies published prior to 25 April 2020. Results: Forty-four papers were chosen for analysis after a detailed inspection, which aimed to keep only the research studies (case control studies and cohort studies) or case reports on children and only those which were written in English. The manifestations of IBD-associated pancreatic disorders range from asymptomatic increase in pancreatic enzymes activity to severe disease such as acute pancreatitis. Acute pancreatitis (AP) induced by drugs, mainly thiopurine, seems to be the most- often-reported pancreatic disease associated with IBD in children. AP associated with other than drug etiologies, and chronic pancreatitis (CP), are rarely observed in the course of pediatric IBD. The pancreatic involvement can be strictly related to the activity of IBD and can also precede the diagnosis of IBD in some pediatric patients. The course of AP is mild in most cases and may occasionally lead to the development of CP, mainly in cases with a genetic predisposition. Conclusions: The involvement of the pancreas in the course of IBD may be considered as an EIM or a separate co-morbid disease, but it can also be a side effect of IBD therapy, therefore a differential diagnosis should always be performed. As the number of IBD incidences with concomitant pancreatic diseases is constantly increasing in the pediatric population, it is important to include pancreatic enzymes level measurement in the workup of IBD.

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“…[ 35 , 37 , 38 ]. Cousin et al also noted the coexistence of severe AIP with rectocolitis, severe liver involvement, in a 16-year-old boy [ 36 ].…”

Section: Resultsmentioning

confidence: 99%

“…Type 2 AIP, on the contrary, is not characterized by elevated IgG4 levels, affects younger patients, and is frequently associated with IBD [ 5 ]. The prevalence of IBD in patients with AIP is higher than in the general population and in most cases UC is diagnosed [ 20 , 35 , 36 , 37 , 38 , 55 ].…”

Section: Discussionmentioning

confidence: 99%

Pancreatic Disorders in Children with Inflammatory Bowel Disease

et al. 2021

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“…Among all 26 studies, 10 [ 13 , 14 , 16 , 18 , 19 , 22 , 23 , 26 , 27 , 29 ], 12 [ 5 , 15 , 17 , 20 , 21 , 25 , 28 , 30 – 32 , 36 , 37 ], and 4 studies [ 24 , 33 – 35 ] were prospective, retrospective, and case reports, respectively. Twenty-one studies [ 5 , 13 – 31 , 34 ] used diagnostic instructions (e.g., International Consensus Diagnostic Criteria, Mayo Clinic’s HiSORT criteria, Japanese Pancreas Society guidelines, or Asian diagnostic criteria).…”

Section: Resultsmentioning

confidence: 99%

The clinical efficacy of azathioprine as maintenance treatment for autoimmune pancreatitis: a systematic review and meta-analysis

et al. 2021

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The effectiveness of azathioprine (AZA) in preventing relapse and maintaining autoimmune pancreatitis (AIP) remission has been reported; however, most of these studies are case series with no randomized control trials available in the literature. Therefore, this study performed a systematic review and meta-analysis of the existing literature on this subject to determine the clinical efficacy of AZA as maintenance therapy for AIP patients. A systematic search was performed to identify studies on the clinical efficacy of AZA as maintenance therapy in AIP patients. The crude multiple relapse rate was estimated to assess the ability of AZA to control relapses in AIP. Pooled estimates were obtained using a random-effects model with the DerSimonian-Laird method. We identified AIP patients who did not respond to initial steroid treatment, experienced steroid weaning failure, or those who relapsed during remission as refractory cases. After reviewing the studies, ten articles fulfilled the inclusion criteria and were selected for meta-analysis. Of all 4504 patients, 3534 patients were treated with steroids, and 346 patients were treated with AZA for relapsed AIP. In this meta-analysis, 14/73 (19.2%) patients receiving AZA for refractory AIP relapsed. Meanwhile, 14/47 (29.8%) patients without AZA experienced relapse. The integrated odds ratio for relapse risk in patients receiving AZA was estimated to be 0.52 (p = 0.15). This systematic review and meta-analysis demonstrated the efficacy of AZA in preventing relapse of AIP, which supports the use of AZA as a maintenance treatment in patients with AIP who relapse upon withdrawal of steroid therapy.

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“…Pediatric AIP can be associated to IBD in up to 25-27% of cases and usually manifests in the form of UC, with the majority of cases represented by type 2 AIP [14,79]. Despite the absence of clear and established diagnostic criteria for its diagnosis [80], data collected suggest that pediatric AIP represents a distinct subtype of pancreatitis that may express histopathological features of both types [65,81,82], including: (i) higher rate of abdominal pain and/or obstructive jaundice at diagnosis, (ii) low rate of high IgG4 levels, (iii) ductal or parenchymal abnormalities on imaging, (iv) lymphoplasmacytic, granulocytic infiltrate with fibrosis, and (v) prompt response to steroid treatment. Recently, the first pediatric AIP recommendation statements have been developed by the "International Study group of Pediatric Pancreatitis: In search for a cure" (INSPPIRE) in order to standardize diagnosis and treatment [83].…”

Section: Autoimmune Pancreatitismentioning

confidence: 99%

Pancreatic Associated Manifestations in Pediatric Inflammatory Bowel Diseases

Cucinotta

Romano

Dipasquale

2021

Genes

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Inflammatory bowel diseases (IBDs) are chronic relapsing inflammatory conditions of the gastrointestinal tract, encompassing Crohn’s disease (CD), ulcerative colitis (UC) and inflammatory bowel disease unclassified (IBD-U). They are currently considered as systemic disorders determined by a set of genetic predispositions, individual susceptibility and environmental triggers, potentially able to involve other organs and systems than the gastrointestinal tract. A large number of patients experiences one or more extraintestinal manifestations (EIMs), whose sites affected are mostly represented by the joints, skin, bones, liver, eyes, and pancreas. Pancreatic abnormalities are not uncommon and are often underestimated, encompassing acute and chronic pancreatitis, autoimmune pancreatitis, exocrine pancreatic insufficiency and asymptomatic elevation of pancreatic enzymes. In most cases they are the result of environmental triggers. However, several genetic polymorphisms may play a role as precipitating factors or contributing to a more severe course. The aim of this paper is to provide an updated overview on the available evidence concerning the etiology, pathogenesis and clinical presentation of pancreatic diseases in IBD pediatric patients.

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Autoimmune Pancreatitis and Ulcerative Rectocolitis in an Adolescent

Cited by 7 publications

References 22 publications

Pancreatic Disorders in Children with Inflammatory Bowel Disease

Pancreatic Disorders in Children with Inflammatory Bowel Disease

The clinical efficacy of azathioprine as maintenance treatment for autoimmune pancreatitis: a systematic review and meta-analysis

Pancreatic Associated Manifestations in Pediatric Inflammatory Bowel Diseases

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