Pediatric Hematology/Oncology and ImmunopathologyPediatric Hematology/Oncology and ImmunopathologyВопросы гематологии/онкологии и иммунопатологии в педиатрии1726-17082414-9314Fund Doctors, Innovations, Science for Children20110.24287/1726-1708-2018-17-3-103-110PROMISING STUDIESПЕРСПЕКТИВНЫЕ ИССЛЕДОВАНИЯTherapy features of interstitial lymphocytic lung disease (ILLD) in patients with immune dysregulation syndromes: case reportОсобенности лечения интерстициальной лимфоцитарной болезни легких у пациентов с синдромами иммунной дизрегуляции: клинический примерhttps://orcid.org/0000-0001-9857-4456RodinaY. A.РодинаЮ. А.
Russian Federation

Julia A. Rodina, MD, allergologisimmunologist of the Immunology Department

117997, Moscow, Samory Mashela st., 1

Родина Юлия Александровна, врач аллерголог-иммунолог, отделение иммунологии 

117997, Москва, ГСП-7, ул. Саморы Машела, 1 

rodina.julija@rambler.ruHorevaA. L.ХореваА. Л.
Russian Federation
AbramovaI. N.АбрамоваИ. Н.
Russian Federation
https://orcid.org/0000-0001-5347-7150ShvetsO. A.ШвецО. А.
Russian Federation
BurlakovV. I.БурлаковВ. И.
Russian Federation
https://orcid.org/0000-0001-7317-7104TereshenkoG. V.ТерещенкоГ. В.
Russian Federation
https://orcid.org/0000-0003-3664-2876AbramovD. S.АбрамовД. С.
Russian Federation
https://orcid.org/0000-0002-9083-4783DeripapaE. V.ДерипапаЕ. В.
Russian Federation
Dmitriy Rogachev National Medical Research Center of Pediatric Hematology, Oncology, ImmunologyФГБУ «Национальный медицинский исследовательский центр детской гематологии, онкологии и иммунологии им. Дмитрия Рогачева»240920181731031102409201824092018Copyright ©; 2018, «D. Rogachev NMRCPHOI»Copyright ©; 2018, ФГБУ «НМИЦ ДГОИ им. Дмитрия Рогачева» Минздрава России2018«D. Rogachev NMRCPHOI»ФГБУ «НМИЦ ДГОИ им. Дмитрия Рогачева» Минздрава Россииhttps://creativecommons.org/licenses/by/4.0https://hemoncim.com/jour/article/view/201

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.

Синдромы дизрегуляции (дефицит генов CTLA4, LRBA) относятся к комбинированным первичным иммунодефицитным состояниям с нарушением аутотолерантности за счет дефекта супрессорной функции Т-регуляторных лимфоцитов и характеризуются развитием тяжелых аутоиммунных осложнений. Одно из них – интерстициальная лимфоцитарная болезнь легких (ИЛБЛ), обусловленная патологической гиперплазией бронхо-ассоциированной лимфоидной ткани, что приводит к потере функции легких, сокращая продолжительность жизни пациента. Диагностика ИЛБЛ основывается на патоморфологическом исследовании легочной ткани и радиологических методах визуализации. Таргетная патогенетическая терапия препаратом абатацепт при синдромах дизрегуляции позволяет достичь максимального контроля над аутоиммунными осложнениями, включая ИЛБЛ, и не обладает значимыми побочными эффектами. В статье представлен клинический разбор пациентов с синдромами дизрегуляции и развитием ИЛБЛ с применением таргетной терапии абатацептом. 

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