IgG4-related disease (IgG4-RD) in developmental age — diagnostic and therapeutic problems
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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
Keywords: IgG4-related disease (IgG4-RD) in childrendiagnosis and therapy
References
- Grygiel-Górniak B, Puszczewicz M. IgG4-related diseases - a new look in rheumatology. Reumatologia/Rheumatology. 2013; 4: 284–292.
- Masiak A, Zdrojewski Z. IgG4-related disease and systemic vasculitis – is there any connection? Reumatologia/Rheumatology. 2014; 6: 384–387.
- Masiak A, Zdrojewski Z. Różne oblicza choroby IgG4-zależnej. Forum Reumatol. 2016; 2(1): 21–31.
- Stone JH, Chan JKC, Deshpande V, et al. IgG4-Related Disease. Int J Rheumatol. 2013; 2013: 532612.
- Pieringer H, Parzer I, Wöhrer A, et al. IgG4- related disease: an orphan disease with many faces. Orphanet J Rare Dis. 2014; 9: 110.
- Umehara H, Okazaki K, Masaki Y, et al. Comprehensive diagnostic criteria for IgG4-related disease (IgG4-RD), 2011. Modern Rheumatology. 2012; 22(1): 21–30.
- Umehara H, Okazaki K, Masaki Y, et al. Research Program for Intractable Disease by Ministry of Health, Labor and Welfare (MHLW) Japan G4 team. A novel clinical entity, IgG4-related disease (IgG4RD): general concept and details. Mod Rheumatol. 2012; 22(1): 1–14.
- Masaki Y, Kurose N, Umehara H. IgG4-related disease: a novel lymphoproliferative disorder discovered and established in Japan in the 21st century. J Clin Exp Hematop. 2011; 51(1): 13–20.
- Okazaki K, Umehara H. Are Classification Criteria for IgG4-RD Now Possible? The Concept of IgG4-Related Disease and Proposal of Comprehensive Diagnostic Criteria in Japan. Int J Rheumatol. 2012; 2012: 357071.
- Corujeira S, Ferraz C, Nunes T, et al. Severe IgG4-Related Disease in a Young Child: A Diagnosis Challenge. Case Rep Pediatr. 2015; 2015: 140753.
- Mannion M, Cron RQ. Successful treatment of pediatric IgG4 related systemic disease with mycophenolate mofetil: case report and a review of the pediatric autoimmune pancreatitis literature. Pediatr Rheumatol Online J. 2011; 9(1): 1.
- Bolia R, Chong SY, Coleman L, et al. Autoimmune Pancreatitis and IgG4 Related Disease in Three Children. ACG Case Rep J. 2016; 3(4): e115.
- Karim F, Loeffen J, Bramer W, et al. IgG4-related disease: a systematic review of this unrecognized disease in pediatrics. Pediatr Rheumatol Online J. 2016; 14(1): 18.
