Autoimmune pancreatitis in an 11-year-old boy

Refaat, Rania; Harth, Marc; Proschek, P.; Lindemayr, Sebastian; Vogl, Thomas J.

doi:10.1007/s00247-008-1132-2

Cited by 21 publications

(13 citation statements)

References 7 publications

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“…Main pancreatic duct irregularities and distal CBD narrowing are nonspecific findings and might also be seen in patients with CP or biliopancreatic diseases of any other etiology or even in primary sclerosing cholangitis type 1 (26), though adults now include this specific type of primary sclerosing cholangitis to the diagnostic criteria for AIP (27). More detailed description of the pancreas and ductal pathology using cross-sectional imaging is challenged by the limitations of the MRCP resolution in young children (14) and the less frequent use of ERCP compared with adults (28). …”

Section: Discussionmentioning

confidence: 99%

Autoimmune Pancreatitis in Children: Characteristic Features, Diagnosis, and Management

Scheers

Palermo

Freedman

et al. 2017

American Journal of Gastroenterology

134

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OBJECTIVES Autoimmune pancreatitis (AIP) is an increasingly recognized disease entity, but data in children are limited. AIP presentation and outcome in children might differ from the adult experience. We aim to determine the characteristic features of AIP in children. METHODS Data about clinical symptoms, imaging, histology, and treatment were collected using two sources: (i) a systematic literature search and (ii) the INSPPIRE database, the largest international multicenter study of pancreatitis in children and the Cliniques Universitaires St-Luc (CUSL) registry. RESULTS We identified 48 AIP cases: 30 from literature review, 14 from INSPPIRE, and 4 from CUSL. The median age at diagnosis was 13 years (range 2–17 years). Abdominal pain (43/47, 91%) and/or obstructive jaundice (20/47, 42%) were the most common symptoms at diagnosis. Elevated serum IgG4 levels were only observed in 9/40 (22%) children. Cross-sectional imaging studies were abnormal in all children including hypointense global or focal gland enlargement (39/47, 83%), main pancreatic duct irregularity (30/47, 64%), and common bile duct stricture (26/47, 55%). A combination of lymphoplasmacytic inflammation, pancreatic fibrosis, and ductal granulocyte infiltration were the main histological findings (18/25, 72%). Children with AIP had a prompt clinical response to steroids. Complications of AIP included failure of exocrine (4/25, 16%) and endocrine (3/27, 11%) pancreas function. CONCLUSIONS Pediatric AIP has a distinct presentation with features similar to type 2 AIP in adults. This comprehensive report on the largest group of children with AIP to date is expected to help with the diagnosis and management of this disease and pave the way for future research studies.

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Section: Discussionmentioning

confidence: 99%

Autoimmune Pancreatitis in Children: Characteristic Features, Diagnosis, and Management

Scheers

Palermo

Freedman

et al. 2017

American Journal of Gastroenterology

134

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“…Only one had normal imaging [14] (she was unable to be diagnosed as AIP by JPS or HISORt criteria, but did respond to steroid therapy and had an elevated IgG4), and 4 of the 7 patients had histology reported, all 4 consistent with AIP [8-10,13]. The only patient with an elevated IgG4 was the patient who did not meet diagnostic criteria for AIP [14]; she also had an elevated total IgG level.…”

Section: Case Presentationmentioning

confidence: 99%

“…The other pediatric patients reported in the literature with AIP or likely AIP (Table 3) were treated with corticosteroids or pancreatoduodenectomy with mixed success. One patient requires daily steroids [12], treatment is not reported in one [10], the surgical patient requires digestive enzymes [13], and one patient requires NPH insulin for diabetes but did not have recurrence of disease [9]. There are many reports of IFP in the literature, but only 4 have either enough criteria information reported or criteria consistent with AIP (Table 3) [15-18].…”

Section: Case Presentationmentioning

confidence: 99%

Successful treatment of pediatric IgG4 related systemic disease with mycophenolate mofetil: case report and a review of the pediatric autoimmune pancreatitis literature

Mannion

Cron

2011

Pediatr Rheumatol

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Autoimmune pancreatitis is frequently associated with elevated serum and tissue IgG4 levels in the adult population, but there are few reports of pediatric autoimmune pancreatitis, and even fewer reports of IgG4 related systemic disease in a pediatric population. The standard of care treatment in adults is systemic corticosteroids with resolution of symptoms in most cases; however, multiple courses of corticosteroids are occasionally required and some patients require long term corticosteroids. In these instances, steroid sparing disease modify treatments are in demand. We describe a 13-year-old girl with IgG4 related systemic disease who presented with chronic recurrent autoimmune pancreatitis resulting in surgical intervention for obstructive hyperbilirubinemia and chronic corticosteroid treatment. In addition, she developed fibrosing medianstinitis as part of her IgG4 related systemic disease. She was eventually successfully treated with mycophenolate mofetil allowing for discontinuation of corticosteroids. This is the first reported use of mycophenolate mofetil for IgG4 related pancreatitis. Although autoimmune pancreatitis as part of IgG4 related systemic disease is rarely reported in pediatrics, autoimmune pancreatitis is also characterized as idiopathic fibrosing pancreatitis. All pediatric autoimmune pancreatitis cases reported in the world medical literature were identified via a PUBMED search and are reviewed herein. Twelve reports of pediatric autoimmune pancreatitis were identified, most of which were treated with corticosteroids or surgical approaches. Most case reports failed to report IgG4 levels, so it remains unclear how commonly IgG4 related autoimmune pancreatitis occurs during childhood. Increased evaluation of IgG4 levels in patients with autoimmune pancreatitis may shed further light on the association of IgG4 with pancreatitis and the underlying pathophysiology.

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“…Since Yoshida et al proposed AIP as a disease entity in 1995, many cases of AIP have been reported in Japan, and AIP has received increasing awareness and better understanding worldwide [2]. The diagnosis of focal forms can be difficult as AIP may mimic pancreatic adenocarcinoma [3,4,5]. Therefore, there have been several cases of AIP in whom resection was performed because of a high suspicion of pancreatic cancer [6, 7].…”

Section: Discussionmentioning

confidence: 99%

Histopathologically Proven Autoimmune Pancreatitis Mimicking Neuroendocrine Tumor or Pancreatic Cancer

Onda¹,

Okamoto²,

Kanehira³

et al. 2012

Case Rep Gastroenterol

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Autoimmune pancreatitis (AIP) can be difficult to distinguish from pancreatic cancer. We report a case of histopathologically proven AIP mimicking neuroendocrine tumor (NET) or pancreatic cancer in a 53-year-old man. He was referred to our hospital for further evaluation of a pancreatic mass detected on ultrasonography at a medical check-up. Abdominal ultrasonography showed a 15-mm hypoechoic mass located in the pancreatic body. Computed tomography revealed a tumor without any contrast enhancement, and magnetic resonance imaging demonstrated the mass to be hyperintense on diffusion-weighted image. Endoscopic retrograde cholangiopancreatography revealed slight dilatation of a branch of the pancreatic duct without stricture of the main pancreatic duct. The common bile duct seemed intact. Under suspicion of a non-functioning NET or malignant neoplasm, laparotomy was performed. At laparotomy, an elastic firm and well-circumscribed mass was found suggestive of a non-functioning NET, thus enucleation was performed. Histopathologically, the lesion corresponded to AIP.

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Autoimmune pancreatitis in an 11-year-old boy

Cited by 21 publications

References 7 publications

Autoimmune Pancreatitis in Children: Characteristic Features, Diagnosis, and Management

Autoimmune Pancreatitis in Children: Characteristic Features, Diagnosis, and Management

Successful treatment of pediatric IgG4 related systemic disease with mycophenolate mofetil: case report and a review of the pediatric autoimmune pancreatitis literature

Histopathologically Proven Autoimmune Pancreatitis Mimicking Neuroendocrine Tumor or Pancreatic Cancer

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