Clinical experience using selective immunomodulating targeted therapy of Interstitial lymphocytic lung disease in a group of pediatric patients with primary immunodeficiencies

Y. A. Rodina; Родина Ю. А.; E. V. Deripapa; Дерипапа Е. В.; O. A. Shvets; Швец О. А.; A. A. Muhina; Мухина А. А.; A. A. Roppelt; Роппельт А. А.; D. V. Yuhacheva; Юхачева Д. В.; A. L. Laberko; Лаберко А. Л.; V. I. Burlakov; Бурлаков В. И.; D. S. Abramov; Абрамов Д. С.; A. B. Golbitc; Гольбиц А. Б.; G. V. Tereshenko; Терещенко Г. В.; A. G. Rumiantsev; Румянцев А. Г.; A. Y. Shcherbina; Щербина А. Ю.

doi:10.24287/1726-1708-2018-17-3-50-59

Clinical experience using selective immunomodulating targeted therapy of Interstitial lymphocytic lung disease in a group of pediatric patients with primary immunodeficiencies

Authors: Rodina Y.A.¹, Deripapa E.V.¹, Shvets O.A.¹, Muhina A.A.¹, Roppelt A.A.¹, Yuhacheva D.V.¹, Laberko A.L.¹, Burlakov V.I.¹, Abramov D.S.¹, Golbitc A.B.², Tereshenko G.V.¹, Rumiantsev A.G.¹, Shcherbina A.Y.¹
Affiliations:
1. Dmitriy Rogachev National Medical Research Center of Pediatric Hematology, Oncology, Immunology Ministry of Healthcare of Russian Federation
2. P. Hertsen Moscow Oncology Research Institute - branch of the National Medical Research Radiological Centre of the Ministry of Health of the Russian Federation
Issue: Vol 17, No 3 (2018)
Pages: 50-59
Section: ORIGINAL ARTICLES
Submitted: 23.09.2018
Accepted: 23.09.2018
Published: 23.09.2018
URL: https://hemoncim.com/jour/article/view/191
DOI: https://doi.org/10.24287/1726-1708-2018-17-3-50-59
ID: 191

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Abstract

Interstitial lymphocytic lung disease (ILLD) is an immune complication of genetically determined primary immunodeficienciencies (PID), resulting from pathological hyperplasia of bronchus–associated lymphoid tissue (BALT) due to the dysregulation of immune system, with progressive loss of pulmonary function. Standard immunosuppressive therapy is not effective in ILLD. Here we describe results of prospective study of efficacy and safety of abatacept and rituximab treatment in a group of 26 children with different forms of PID (median of age 12,1years). Therapeutic drug was chosen based on patterns of lymphocytic lung infiltration - with predominantly T- or B–cell content. We demonstrate, that targeted therapy with abatacept and rituximab not only allows to achieve remission of clinical and radiological ILLD’s symptoms in both groups, but also significant improvements quality of life of patients (57 ± 2.1 vs 31.2 ± 1.9 points; p < 0,01).

Keywords

interstitial lung disease, primary immunodeficiency, children, therapy, rituximab, abatacept, quality of life

About the authors

Y. A. Rodina

Dmitriy Rogachev National Medical Research Center of Pediatric Hematology, Oncology, Immunology Ministry of Healthcare of Russian Federation

Author for correspondence.
Email: rodina.julija@rambler.ru
ORCID iD: 0000-0001-9857-4456

Julia A. Rodina, MD, allergologist– immunologist of the Immunology Department

117997, Moscow, Samory Mashela st., 1

Russian Federation

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