{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,18]],"date-time":"2026-08-18T04:14:39Z","timestamp":1787026479320,"version":"build-2736575974"},"reference-count":32,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2021,9,9]],"date-time":"2021-09-09T00:00:00Z","timestamp":1631145600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["frontiersin.org"],"crossmark-restriction":true},"short-container-title":["Front. Immunol."],"abstract":"<jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>Interstitial lymphocytic lung disease (ILLD), a recently recognized complication of primary immunodeficiencies (PID), is caused by immune dysregulation, abnormal bronchus-associated lymphoid tissue (BALT) hyperplasia, with subsequent progressive loss of pulmonary function. Various modes of standard immunosuppressive therapy for ILLD have been shown as only partially effective.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Objectives<\/jats:title>\n                    <jats:p>To retrospectively evaluate the safety and efficacy of abatacept or rituximab in treatment of ILLD in children with PID.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>29 children (median age 11 years) with various forms of PID received one of the two therapy regimens predominantly based on the lesions\u2019 immunohistopathology: children with prevalent B-cell lung infiltration received rituximab (n = 16), and those with predominantly T-cell infiltration received abatacept (n = 17). Clinical and radiological symptoms were assessed using a severity scale developed for the study.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      The targeted therapy with abatacept (A) or rituximab (R) enabled long-term control of clinical (A 3.4 \u00b1 1.3\n                      <jats:italic>vs.<\/jats:italic>\n                      0.6 \u00b1 0.1; R 2.8 \u00b1 1\n                      <jats:italic>vs.<\/jats:italic>\n                      0.7 \u00b1 0.05, p &amp;lt; 0.01) and radiological (A 18.4 \u00b1 3.1\n                      <jats:italic>vs.<\/jats:italic>\n                      6.0 \u00b1 2.0; R 30 \u00b1 7.1\n                      <jats:italic>vs.<\/jats:italic>\n                      10 \u00b1 1.7, p &amp;lt; 0.01) symptoms of ILLD in both groups and significantly improved patients\u2019 quality of life, as measured by the total scale (TS) score of 57 \u00b1 2.1 in treatment recipients\n                      <jats:italic>vs.<\/jats:italic>\n                      31.2 \u00b1 1.9 before therapy (p &amp;lt; 0.01).\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>ILLD histopathology should be considered when selecting treatment. Abatacept and rituximab are effective and safe in differential treatment of ILLD in children.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.3389\/fimmu.2021.704261","type":"journal-article","created":{"date-parts":[[2021,9,9]],"date-time":"2021-09-09T05:15:02Z","timestamp":1631164502000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":8,"title":["Rituximab and Abatacept Are Effective in Differential Treatment of Interstitial Lymphocytic Lung Disease in Children With Primary 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