Email this article Clinical case of IgG4-related disease
- Authors: Sagoyan G.B.1, Abramov D.S.1, Deordieva E.A.1, Burlakov V.I.1, Tereshchenko G.V.1, Kozlova A.L.1
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Affiliations:
- Dmitriy Rogachev National Medical Research Center of Pediatric Hematology, Oncology, Immunology
- Issue: Vol 17, No 2 (2018)
- Pages: 114-120
- Section: ШКОЛА ИММУНОЛОГА
- Submitted: 20.09.2018
- Published:
- URL: https://hemoncim.com/jour/article/view/183
- DOI: https://doi.org/10.24287/1726-1708-2018-17-2-114-120
- ID: 183
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Abstract
IgG4-related disease is an extremely rare disorder, сcharacterized by the inflammatory infiltration of various organs, with predominance of IgG4-positive plasma cells, fibrosis and high IgG4 plasma concentration. Treatment regiments have not been optimized, especially in children. Here we discuss a case of IgG4-related disease of the orbit in a 13 year old boy and our new treatment approach involving JAK-kinase inhibitor ruxolitininb. The article also presents analysis of the state of the problem of IgG4-related disease it he world, including terminology, historical references, pathogenesis, diagnostic problems and clinical manifestations.
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About the authors
G. B. Sagoyan
Dmitriy Rogachev National Medical Research Center of Pediatric Hematology, Oncology, Immunology
Author for correspondence.
ORCID iD: 0000-0002-7846-3473
Russian Federation
D. S. Abramov
Dmitriy Rogachev National Medical Research Center of Pediatric Hematology, Oncology, Immunology
ORCID iD: 0000-0003-3664-2876
Russian Federation
E. A. Deordieva
Dmitriy Rogachev National Medical Research Center of Pediatric Hematology, Oncology, Immunology
ORCID iD: 0000-0002-8208-2075
Russian Federation
V. I. Burlakov
Dmitriy Rogachev National Medical Research Center of Pediatric Hematology, Oncology, ImmunologyRussian Federation
G. V. Tereshchenko
Dmitriy Rogachev National Medical Research Center of Pediatric Hematology, Oncology, Immunology
ORCID iD: 0000-0001-7317-7104
Russian Federation
A. L. Kozlova
Dmitriy Rogachev National Medical Research Center of Pediatric Hematology, Oncology, Immunology
Email: annamax-99@mail.ru
ORCID iD: 0000-0002-2869-6535
MD, PhD, Department of immunology
Russia 117997, Moscow, Samory Mashela st., 1
Russian FederationReferences
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