Year: 2019
Type: article
Study design: Case Report
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
Source: Forum Reumatologiczne
DOI: 10.5603/fr.2019.0017
Authors: Justyna Orlicka, Dorota Turowska-Heydel, Zbigniew Michał Żuber, Prof. KA AFM (Kraków)
Institution: Uniwersytecki Szpital Dziecięcy
Language: English
Subfield: Rheumatology
Field: Medicine
Domain: Health Sciences
Open Access status: diamond
OpenAlex ID: W2974708564