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Pediatric Rheumatology
Published in: Pediatric Rheumatology 1/2016

Open Access 01-12-2016 | Review

IgG4-related disease: a systematic review of this unrecognized disease in pediatrics

Authors: Faiz Karim, Jan Loeffen, Wichor Bramer, Lauren Westenberg, Rob Verdijk, Martin van Hagen, Jan van Laar

Published in: Pediatric Rheumatology | Issue 1/2016

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Abstract

Background

Immunoglobulin G4-related disease (IgG4-RD) is a systemic fibro-inflammatory condition with an unclear pathophysiological mechanism affecting different parts of the body. If untreated, the disease can lead to fibrosis and irreversible organ damage. IgG4-RD mostly has been described in adults, hence it is generally unknown among pediatricians. This systematic search of the literature provides an overview of all reports published on IgG4-RD in children in order to create awareness of IgG4-RD in pediatrics and to emphasize the broad clinical presentation of this disease.

Methods

A systematic literature search of Embase, Medline, Web-of-Science, PubMed publisher, Cochrane and Google Scholar was performed for case reports on IgG4-RD in children.

Results

Of total 740 articles identified by the search, 22 case reports including 25 cases of IgG4-RD in children were found. The median age of the children was 13 years, of which 64 % were girls. IgG4-related orbital disease (44 %) and autoimmune pancreatitis type 1/IgG4-related pancreatitis (12 %) predominantly occurred. Less frequently, other manifestations as pulmonary manifestation, cholangitis and lymphadenopathy were also found. Almost all cases were histologically proven. Prednisone was the first choice of treatment leading to favorable clinical response in 83 % of the cases. Maintenance therapy with steroid sparing agents was required in 43 % of the cases needing therapy. Rituximab was successful in all 4 cases, whereas, the disease modifying rheumatic drugs (DMARDs) mycophenolate mofetil, azathioprine and methotrexate were effective in almost 50 % of the cases.

Conclusion

IgG4-RD in children is a generally unknown disease among pediatricians, but several pediatric cases have been described. Prednisone is the first choice of treatment leading to disease remission in the majority of the cases. DMARDs and rituximab are alternative effective steroid sparing agents with more positive evidence for the latter.
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Metadata
Title
IgG4-related disease: a systematic review of this unrecognized disease in pediatrics
Authors
Faiz Karim
Jan Loeffen
Wichor Bramer
Lauren Westenberg
Rob Verdijk
Martin van Hagen
Jan van Laar
Publication date
01-12-2016
Publisher
BioMed Central
Published in
Pediatric Rheumatology / Issue 1/2016
Electronic ISSN: 1546-0096
DOI
https://doi.org/10.1186/s12969-016-0079-3

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