Autoimmune pancreatitis (AIP) is a rare entity that is extremely uncommon in children. Its diagnosis is also a clinical challenge. This form of chronic pancreatitis often presents itself with obstructive jaundice and/or a pancreatic mass and it is sometimes misdiagnosed as pancreatic cancer. We describe the case of a 13-year-old boy with obstructive jaundice and a 4 cm mass in the head of the pancreas that was diagnosed as AIP with associated ulcerative colitis.info:eu-repo/semantics/publishedVersio

Cabral, J

Campos, AP

Gouveia, CI

Oliveira, L

English

Repositório do Centro Hospitalar de Lisboa Central, EPE

1Gouveia CI, et al. BMJ Case Rep 2018;11:e227888. doi:10.1136/bcr-2018-227888Case reportAutoimmune pancreatitis with associated ulcerative colitis in a teenagerCarolina Isabel Gouveia,1 Laura oliveira,2 antónio p Campos,2 José Cabral2Rare diseaseTo cite: Gouveia CI, oliveira L, Campos ap, et al. BMJ Case Rep 2018;11:e227888. doi:10.1136/bcr-2018-2278881Department of pediatric, Hospital Doutor Nelio Mendonca, Funchal, portugal2Department of paediatric Gastroenterology Unit, Hospital Dona estefania, Lisboa, portugalCorrespondence toDr Carolina Isabel Gouveia,   carolinaifgouveia@ gmail. comaccepted 24 November 2018© BMJ publishing Group Limited 2018. No commercial re-use. see rights and permissions. published by BMJ.Summaryautoimmune pancreatitis (aIp) is a rare entity that is extremely uncommon in children. Its diagnosis is also a clinical challenge. this form of chronic pancreatitis often presents itself with obstructive jaundice and/or a pancreatic mass and it is sometimes misdiagnosed as pancreatic cancer. We describe the case of a 13-year-old boy with obstructive jaundice and a 4 cm mass in the head of the pancreas that was diagnosed as aIp with associated ulcerative colitis.BaCkground Autoimmune pancreatitis (AIP) is an autoimmune disorder that occurs primarily in adults and it is extremely rare in children. This form of chronic pancreatitis may resemble pancreatic neoplasia and is frequently associated with obstructive jaundice, with or without a pancreatic mass.1–3 Distinguishing between these two entities can prove challenging. While diagnostic criteria and management strat-egies for AIP have been developed for adults, no diagnostic criteria have been established for chil-dren. A current report established the character-istic features of paediatric autoimmune pancreatitis (P-AIP) based on a review of all children with AIP to date and, more recently, the same panel of experts developed recommendation statements to stan-dardise the diagnostic and management approach of these patients.3 4 As such, we report on this case to increase knowledge about this rare entity in the paediatric population.CaSe preSenTaTionA 13-year-old boy presented with a 3-month history of asthenia, weight loss, abdominal pain and progressive obstructive jaundice 10 days before his admission to the hospital. Labora-tory evaluation revealed elevated hepatobiliary enzymes, elevated total and direct bilirubin, sevenfold increased lipase and slightly elevated amylase. Viral panel was negative. Cancer antigen (CA) 19-9 levels were elevated and anti-nuclear antibody was positive (1/60). Alpha-feto-protein and other autoantibodies were negative. Immunoglobulin levels were normal but subclass IgG4 was elevated twofold (table 1).Abdominal ultrasound, abdominal MRI and MR cholangiopancreatography (MRCP) revealed a 4 cm mass in the pancreatic head (figure 1), causing common bile duct (CBD) obstruction with significant intrahepatic and extrahepatic bile duct dilatation, distended gallbladder and hepato-megaly (figure 2). Endoscopic ultrasound with fine needle aspiration (EUS-FNA) revealed an enlarged pancreatic head and atrophic tail, suggestive of a pseudo-mass and regular CBD dilatation. Biopsy samples were insufficient for diagnosis. An endo-scopic retrograde cholangiopancreatography (ERCP) with stent placement was performed, with clinical and laboratory improvement.A second EUS-FNA showed pancreatic ducts and acini with lymphoplasmacytic infiltrate and some fibrosis patches that were compatible with an inflammatory process. There was no evidence of neoplastic cells. IgG4 stain was also negative. After ruling out malignancy, the diagnosis of AIP was considered. Because there was a significant clinical and laboratory improvement with reduction of the pancreatic size after resolving biliary obstruction, treatment with steroids was not started.ouTCome and follow-upIn the following months, there was a complete normalisation of the laboratory findings, apart for the IgG4 levels which remained elevated. A follow-up MRCP showed significant reduction of the pancreatic head and a subtle dilatation of the intrahepatic and extrahepatic bile ducts. A second ERCP was performed with new stent placement due to distal CBD stenosis. The stent was removed 16 weeks later with complete resolution of pancre-atic enlargement and of biliary and pancreatic ducts dilatation. Although asymptomatic, the patient underwent colonoscopy a few months after AIP diagnosis due elevated calprotectin levels and recognised association between AIP and inflamma-tory bowel disease.Ulcerative colitis (UC) was then diagnosed. Corti-costeroid treatment was started and azathioprine was subsequently added.A year later, the patient had a relapse of UC with the need to undergo therapeutic escalation with infliximab. Regardless, his AIP remained in remis-sion at 3½ years of follow-up.diSCuSSionAlthough AIP is a well-known disease in adults, the experience in children is limited to a few cases described in existing literature.1 3 5–9 Therefore, due to the disease’s rarity in children, the diagnosis of AIP in paediatric population requires a high index of suspicion.2 Gouveia CI, et al. BMJ Case Rep 2018;11:e227888. doi:10.1136/bcr-2018-227888rare diseaseUntil recently there were no established guidelines in diagnosis and management for AIP in children and the approach was based on adult criteria.2 However, it has been suggested that clinical presentation of AIP may be different in children and adults. As such, a recent report, which was based on data collected from previously reported cases and a large paediatric multicentre cohort study of pancreatitis (International Study Group of Pedi-atric Pancreatitis: In Search for a Cure—INSPPIRE), attempted to establish the characteristic features of AIP in children.3 More recently, the same group of paediatric gastroenterologists devel-oped clinical recommendations for the definition, diagnosis and treatment approach of P-AIP.4Most children with AIP present with abdominal pain and/or obstructive jaundice in association with focal pancreatic enlargement, main pancreatic duct irregularities and distal CBD narrowing.1 3–9 As in adults, there is a clinical challenge in distin-guishing a neoplastic condition from AIP, since they benefit from different treatment approaches.Two distinct subtypes of AIP have been identified in adults, each with differing associations, clinical courses, clinical profiles and histopathological patterns: type 1 or lymphop-lasmacytic sclerosing pancreatitis and type 2 or idiopathic duct-centric pancreatitis.2 It is now clear that P-AIP is a distinct subtype of pancreatitis that may present with histopatho-logical features of both types. The pancreatic parenchymal changes in P-AIP includes lymphoplasmacytic and/or neutro-philic infiltrates and/or parenchymal fibrosis.4 Even though histopathological findings are used as a diagnostic criterion for AIP, considering the barriers in obtaining pancreatic biop-sies in children, P-AIP diagnosis may be based on clinical and imaging findings.3 4High levels of serum IgG4 may be suggestive of AIP in adults, specially of type 1, however this marker has limited value in chil-dren and can be non-specifically elevated in patients with other conditions, as well.6 10While a clear association exists between AIP and concurrent immune/inflammatory diseases, especially UC, their exact rela-tionship is still unclear.4 5The imaging, clinical, analytical and histological evaluations in this case were indicative of P-AIP associated with UC. Pancre-atic head enlargement and distal CBD narrowing associated with weight loss, abdominal pain and obstructive jaundice were present. The histological examination was negative for malig-nant cells and consistent with an inflammatory process, with no evidence of IgG4 plasma cells on immunostaining.The current standard treatment for P-AIP is steroid therapy, the same as adults. In some P-AIP cases, the main clinical finding for the disease diagnosis has been steroid-responsive pancreatic enlargement.6 7 However, the current case saw a spontaneous regression of the pancreatic mass after relief of the biliary obstruction. Some reports have described spontaneous resolu-tion of pancreatic masses, jaundice and strictures in the context of AIP, and some P-AIP patients have experienced disease resolu-tion without any treatment.3 4 11 12 In this case it was licit to have a wait-and-see approach based on the rapid clinical response and, so far, the patient has not relapsed.Table 1 Laboratory data on admission (normal range)Leucocytes, ×109/L 11.2Haemoglobin, g/dL 14.3Platelets, ×1010/L 408CPR (<5 mg/L) 9.5ESR (mm/hour) 40Lipase (U/L) (4–39) 295Amylase (U/L) (8–51) 51AST (U/L) (14–35) 125ALT (U/L) (8–24) 330Total bilirubin (mg/dL) (0.3–1.2) 7.91Direct bilirubin (mg/dL) 4.62GGT (U/L) (7–21) 406Alkaline phosphatase (U/L) (127–517) 756IgG (mg/L) (840–2013) 1470IgG2 (mg/L) (140–440) 555IgG4 (mg/L) (1–83) 268Faecal elastase (<200 µg/g) <30AFP (ng/mL) (0.6–3.9) 0.8CA 19.9 (U/mL) (<35) 192.7AFP, alpha-fetoprotein; ALT, alanine aminotransferase; AST, aspartate aminotransferase; CRP, C-reactive protein; ESR, erythrocyte sedimentation rate; GGT, γ-glutamyl transferase.figure 1 Axial T2-weighted abdominal MRI showing enlargement of the pancreatic head (arrows).figure 2 MR cholangiopancreatography (MRCP) showing narrowed distal common bile duct with markedly dilated intrahepatic and extrahepatic ducts.3Gouveia CI, et al. BMJ Case Rep 2018;11:e227888. doi:10.1136/bcr-2018-227888rare diseaseContributors CIFG: wrote the main manuscript and was involved in literature search; Lo, apC and JC were actively involved in the patient care and all assisted in revision of the manuscript.funding the authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors. Competing interests None declared.patient consent for publication obtained.provenance and peer review Not commissioned; externally peer reviewed.RefeRences 1 Friedlander J, Quiros Ja, Morgan t, et al. Diagnosis of autoimmune pancreatitis vs neoplasms in children with pancreatic mass and biliary obstruction. Clin Gastroenterol Hepatol 2012;10:1051–5. 2 shimosegawa t, Chari st, Frulloni L, et al. International consensus diagnostic criteria for autoimmune pancreatitis: guidelines of the international association of pancreatology. Pancreas 2011;40:352–8. 3 scheers I, palermo JJ, Freedman s, et al. autoimmune pancreatitis in children: characteristic features, diagnosis, and management. Am J Gastroenterol 2017;112:1604–11. 4 scheers I, palermo JJ, Freedman s, et al. recommendations for diagnosis and management of autoimmune pancreatitis in childhood: consensus from INsppIre. J Pediatr Gastroenterol Nutr 2018;67:232–6. 5 roque ramos L, DiMaio CJ, sachar DB, et al. autoimmune pancreatitis and inflammatory bowel disease: Case series and review of the literature. Dig Liver Dis 2016;48:893–8. 6 Zen Y, Grammatikopoulos t, Hadzic N. autoimmune pancreatitis in children: insights into the diagnostic challenge. J Pediatr Gastroenterol Nutr 2014;59:e42–e452014. 7 Long J, Birken G, Migicovsky B. autoimmune pancreatitis in a child presenting as a pancreatic mass. J Pediatr Surg Case Rep 2015;3:111–3. 8 Bolia r, Chong sY, Coleman L, et al. autoimmune pancreatitis and IgG4 related disease in three children. ACG Case Rep J 2016;3:e115. 9 Gargouri L, ponsot p, Viala J, et al. recurrent autoimmune pancreatitis in a 10-year-old boy. J Pediatr Gastroenterol Nutr 2009;48:374–7. 10 Ghazale a, Chari st, smyrk tC, et al. Value of serum IgG4 in the diagnosis of autoimmune pancreatitis and in distinguishing it from pancreatic cancer. Am J Gastroenterol 2007;102:1646–53. 11 Kamisawa t, Yoshiike M, egawa N, et al. treating patients with autoimmune pancreatitis: results from a long-term follow-up study. Pancreatology 2005;5:234–40. 12 Kamisawa t, takeuchi t. treatment of autoimmune pancreatitis with the anecdotes of the first report. Int J Rheumatol 2012;2012:1–4.learning points ► Autoimmune pancreatitis (AIP) rarely occurs in children and is a distinctive subtype of pancreatitis. ► Paediatric autoimmune pancreatitis (P-AIP) should be taken into consideration in the differential diagnosis in children with a pancreatic enlargement. ► Most children with AIP present with abdominal pain and/or obstructive jaundice. ► The diagnosis of P-AIP can be made based on the combination of clinical presentation and imaging findings, possibly complemented by histopathological findings. ► First choice treatment in P-AIP are steroids. However, some patients may have symptom resolution without any treatment.Copyright 2018 BMJ publishing Group. all rights reserved. For permission to reuse any of this content visithttps://www.bmj.com/company/products-services/rights-and-licensing/permissions/BMJ Case report Fellows may re-use this article for personal use and teaching without any further permission.Become a Fellow of BMJ Case reports today and you can: ► submit as many cases as you like ► enjoy fast sympathetic peer review and rapid publication of accepted articles ► access all the published articles ► re-use any of the published material for personal use and teaching without further permissionFor information on Institutional Fellowships contact consortiasales@bmjgroup.comVisit casereports.bmj.com for more articles like this and to become a Fellow

Autoimmune Pancreatitis with Associated Ulcerative Colitis in a Teenager

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Autoimmune Pancreatitis with Associated Ulcerative Colitis in a Teenager

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