Email this article Therapy features of interstitial lymphocytic lung disease (ILLD) in patients with immune dysregulation syndromes: case report
- Authors: Rodina Y.A.1, Horeva A.L.1, Abramova I.N.1, Shvets O.A.1, Burlakov V.I.1, Tereshenko G.V.1, Abramov D.S.1, Deripapa E.V.1
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Affiliations:
- Dmitriy Rogachev National Medical Research Center of Pediatric Hematology, Oncology, Immunology
- Issue: Vol 17, No 3 (2018)
- Pages: 103-110
- Section: PROMISING STUDIES
- Submitted: 24.09.2018
- Accepted: 24.09.2018
- Published: 24.09.2018
- URL: https://hemoncim.com/jour/article/view/201
- DOI: https://doi.org/10.24287/1726-1708-2018-17-3-103-110
- ID: 201
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Abstract
Immune dysregulation syndromes (CTLA4 and LRBA deficiency) belong to the combined primary immunodeficiencies with defective tolerance due to lack of T regulatory lymphocytes function and resulting autoimmune complications. One of them is interstitial lymphocytic lung disease (ILLD), caused by pathologic bronchi-associated lymphoid tissue hyperplasia, progressive loss of lung function and decreased life expectancy. ILLD diagnosis is based on pathomorphological lung changes and radiologic symptoms. Targeted therapy with Abatacept in patients with immune dysregulation syndromes provides control of the autoimmune complications, including ILLD and is well tolerated. Here we demonstrate two cases of patients with immune dysregulation syndromes and ILLD treated with Abatacept.
About the authors
Y. A. Rodina
Dmitriy Rogachev National Medical Research Center of Pediatric Hematology, Oncology, Immunology
Author for correspondence.
Email: rodina.julija@rambler.ru
ORCID iD: 0000-0001-9857-4456
Julia A. Rodina, MD, allergologisimmunologist of the Immunology Department
117997, Moscow, Samory Mashela st., 1
Russian FederationA. L. Horeva
Dmitriy Rogachev National Medical Research Center of Pediatric Hematology, Oncology, ImmunologyRussian Federation
I. N. Abramova
Dmitriy Rogachev National Medical Research Center of Pediatric Hematology, Oncology, ImmunologyRussian Federation
O. A. Shvets
Dmitriy Rogachev National Medical Research Center of Pediatric Hematology, Oncology, Immunology
ORCID iD: 0000-0001-5347-7150
Russian Federation
V. I. Burlakov
Dmitriy Rogachev National Medical Research Center of Pediatric Hematology, Oncology, ImmunologyRussian Federation
G. V. Tereshenko
Dmitriy Rogachev National Medical Research Center of Pediatric Hematology, Oncology, Immunology
ORCID iD: 0000-0001-7317-7104
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. V. Deripapa
Dmitriy Rogachev National Medical Research Center of Pediatric Hematology, Oncology, Immunology
ORCID iD: 0000-0002-9083-4783
Russian Federation
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