Autoimmune pancreatitis in children: A single centre experience in diagnosis, management and long term follow up.
Autoimmune pancreatitis
Children
Diagnosis
Granulocyte epithelial lesion
Immunoglobulin G subclass 4
Journal
Pancreatology : official journal of the International Association of Pancreatology (IAP) ... [et al.]
ISSN: 1424-3911
Titre abrégé: Pancreatology
Pays: Switzerland
ID NLM: 100966936
Informations de publication
Date de publication:
Jan 2019
Jan 2019
Historique:
received:
14
06
2018
revised:
10
11
2018
accepted:
12
11
2018
pubmed:
21
11
2018
medline:
23
4
2019
entrez:
21
11
2018
Statut:
ppublish
Résumé
Autoimmune pancreatitis (AIP) is a rare form of chronic pancreatitis and data is limited in the paediatric population. We aim to describe in detail a cohort of paediatric patients with AIP including their presentation, investigations that led to their diagnosis, management and long-term follow up. We retrospectively reviewed the data of 6 patients diagnosed with AIP over an 10-year period. Data including demographics, clinical information, laboratory parameters, serological markers, radiological and histological findings as well as longitudinal follow up were collected. Out of the six patients, one was diagnosed with definitive Type 1 AIP, two with definitive Type 2 AIP, two with probable Type 2 AIP and one with suspected Type 2 AIP. Median time of follow up was 3.9 years (range 2.6-10.1). 4 patients had pancreatic biopsies with 2 of these patients showing granulocytic epithelial lesions (GELs). 4 patients received steroids and two of them developed ulcerative colitis. Azathioprine was commenced on the patient with Type 1 AIP to help her wean off steroids that caused significant side effects on her. Only two patients developed exocrine insufficiency. The long term follow up of our cohort of paediatric AIP shows good prognosis. More follow up data on patients with AIP is needed to help further characterize and define the disease.
Identifiants
pubmed: 30455055
pii: S1424-3903(18)30751-8
doi: 10.1016/j.pan.2018.11.004
pii:
doi:
Types de publication
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Pagination
169-176Informations de copyright
Crown Copyright © 2018. Published by Elsevier B.V. All rights reserved.