open access

Vol 5, No 3 (2019)
Przypadki kliniczne
Published online: 2019-09-19
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IgG4-related disease (IgG4-RD) in developmental age — diagnostic and therapeutic problems

Justyna Orlicka, Dorota Turowska-Heydel, Zbigniew Michał Żuber, Prof. KA AFM (Kraków)
DOI: 10.5603/FR.2019.0017
·
Forum Reumatologiczne 2019;5(3):149-153.

open access

Vol 5, No 3 (2019)
Przypadki kliniczne
Published online: 2019-09-19

Abstract

The IgG4-related disease (IgG4-RD) is a type of a Hypergammaglobulinemia with elevated class IgG4 of unknown aetiology. It is still rarely diagnosed due to lack of clear diagnostic criteria and uncertain ethiopathogenesis. The most characteristic features are lymphocytes and plasmocytes IgG4+ inflammatory infiltrations located in various tissues and organs with elevated blood serum levels of IgG4. It mostly affects lacrimal glands, salivary glands and pancreas, therefore the clinical picture may suggest the Sjögren syndrome. The inflammatory process that is chronic and reccurent finally leads to fibrosis and sclerosis of affected tissues and organs function deterioration. The clinical picture of IgG4-RD is highly variable depending on which organs are affected. Most patients may be treated with glycocortykosteroids, however some of them develop recrudescence. The article consists of case report of the 11-yearsold boy diagnosed with the IgG4-RD. The authors showed the diagnostic and therapeutic process paying particular attention to avoid diagnostic traps. They discussed the diagnostic criteria and differential
diagnostics. The IgG4-RD among pediatric population is still barely known by paediatricians. Only isolated cases are described so far.

Forum Reumatol. 2019, tom 5, nr 3: 149–153

Abstract

The IgG4-related disease (IgG4-RD) is a type of a Hypergammaglobulinemia with elevated class IgG4 of unknown aetiology. It is still rarely diagnosed due to lack of clear diagnostic criteria and uncertain ethiopathogenesis. The most characteristic features are lymphocytes and plasmocytes IgG4+ inflammatory infiltrations located in various tissues and organs with elevated blood serum levels of IgG4. It mostly affects lacrimal glands, salivary glands and pancreas, therefore the clinical picture may suggest the Sjögren syndrome. The inflammatory process that is chronic and reccurent finally leads to fibrosis and sclerosis of affected tissues and organs function deterioration. The clinical picture of IgG4-RD is highly variable depending on which organs are affected. Most patients may be treated with glycocortykosteroids, however some of them develop recrudescence. The article consists of case report of the 11-yearsold boy diagnosed with the IgG4-RD. The authors showed the diagnostic and therapeutic process paying particular attention to avoid diagnostic traps. They discussed the diagnostic criteria and differential
diagnostics. The IgG4-RD among pediatric population is still barely known by paediatricians. Only isolated cases are described so far.

Forum Reumatol. 2019, tom 5, nr 3: 149–153

Get Citation

Keywords

IgG4-related disease (IgG4-RD) in children; diagnosis and therapy

About this article
Title

IgG4-related disease (IgG4-RD) in developmental age — diagnostic and therapeutic problems

Journal

Forum Reumatologiczne

Issue

Vol 5, No 3 (2019)

Pages

149-153

Published online

2019-09-19

DOI

10.5603/FR.2019.0017

Bibliographic record

Forum Reumatologiczne 2019;5(3):149-153.

Keywords

IgG4-related disease (IgG4-RD) in children
diagnosis and therapy

Authors

Justyna Orlicka
Dorota Turowska-Heydel
Zbigniew Michał Żuber, Prof. KA AFM (Kraków)

References (13)
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