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IgG4-related disease (IgG4-RD) in developmental age — diagnostic and therapeutic problems
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Affiliations- Oddział Dzieci Starszych z Pododdziałami Neurologii i Reumatologii Szpitala Dziecięcego im. św. Ludwika w Krakowie
- Katedra Pediatrii KA AFM Kraków, G. Herlinga-Grudzińskiego 1, 30-750 Kraków, Poland
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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
Keywords
IgG4-related disease (IgG4-RD) in children; diagnosis and therapy
About this articleTitle
IgG4-related disease (IgG4-RD) in developmental age — diagnostic and therapeutic problems
Journal
Issue
Article type
Case report
Pages
149-153
Published online
2019-09-19
Page views
673
Article views/downloads
1161
DOI
Bibliographic record
Forum Reumatol 2019;5(3):149-153.
Keywords
IgG4-related disease (IgG4-RD) in children
diagnosis and therapy
Authors
Justyna Orlicka
Dorota Turowska-Heydel
Zbigniew Żuber
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