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Rituximab Monotherapy Is Effective as First-Line Treatment for Granulomatous Lymphocytic Interstitial Lung Disease (GLILD) in CVID Patients
Granulomatous lymphocytic interstitial lung disease (GLILD) represents a fatal immune dysregulatory complication in common variable immunodeficiency (CVID). Evidence-based diagnostic guidelines are lacking, and GLILD treatment consists in immunosuppressive drugs; nonetheless, therapeutical strategie...
Autores principales: | , , , , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Springer US
2023
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10661825/ https://www.ncbi.nlm.nih.gov/pubmed/37755605 http://dx.doi.org/10.1007/s10875-023-01587-4 |
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author | Tessarin, Giulio Baronio, Manuela Gazzurelli, Luisa Rossi, Stefano Chiarini, Marco Moratto, Daniele Giliani, Silvia Clara Bondioni, Maria Pia Badolato, Raffaele Lougaris, Vassilios |
author_facet | Tessarin, Giulio Baronio, Manuela Gazzurelli, Luisa Rossi, Stefano Chiarini, Marco Moratto, Daniele Giliani, Silvia Clara Bondioni, Maria Pia Badolato, Raffaele Lougaris, Vassilios |
author_sort | Tessarin, Giulio |
collection | PubMed |
description | Granulomatous lymphocytic interstitial lung disease (GLILD) represents a fatal immune dysregulatory complication in common variable immunodeficiency (CVID). Evidence-based diagnostic guidelines are lacking, and GLILD treatment consists in immunosuppressive drugs; nonetheless, therapeutical strategies are heterogeneous and essentially based on experts’ opinions and data from small case series or case reports. We aimed to evaluate the efficacy and safety of first-line Rituximab monotherapy for CVID-related GLILD, by assessing symptoms and quality of life alterations, immunological parameters, pulmonary function tests, and lung computed tomography. All six GLILD patients received Rituximab infusions as a first-line treatment. Rituximab was administered at 375 mg/m(2) monthly for six infusions followed by maintenance every 3 months; none of the patients experienced severe adverse events. Symptom burden and quality of life significantly improved in treated patients compared to a control group of CVID patients without GLILD. Rituximab treatment indirectly caused a trend toward reduced T-cell activation and exhaustion markers sCD25 and sTIM-3. Lung function improved in treated patients, with statistically significant increases in TLC and DL(CO). Lung CT scan findings expressed by means of Baumann scoring system displayed a reduction in the entire cohort. In conclusion, first-line monotherapy with Rituximab displayed high efficacy in disease remission in all treated patients, with improvement of symptoms and amelioration of quality of life, as well as restoration of PFTs and lung CT scan findings. SUPPLEMENTARY INFORMATION: The online version contains supplementary material available at 10.1007/s10875-023-01587-4. |
format | Online Article Text |
id | pubmed-10661825 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2023 |
publisher | Springer US |
record_format | MEDLINE/PubMed |
spelling | pubmed-106618252023-09-27 Rituximab Monotherapy Is Effective as First-Line Treatment for Granulomatous Lymphocytic Interstitial Lung Disease (GLILD) in CVID Patients Tessarin, Giulio Baronio, Manuela Gazzurelli, Luisa Rossi, Stefano Chiarini, Marco Moratto, Daniele Giliani, Silvia Clara Bondioni, Maria Pia Badolato, Raffaele Lougaris, Vassilios J Clin Immunol Original Article Granulomatous lymphocytic interstitial lung disease (GLILD) represents a fatal immune dysregulatory complication in common variable immunodeficiency (CVID). Evidence-based diagnostic guidelines are lacking, and GLILD treatment consists in immunosuppressive drugs; nonetheless, therapeutical strategies are heterogeneous and essentially based on experts’ opinions and data from small case series or case reports. We aimed to evaluate the efficacy and safety of first-line Rituximab monotherapy for CVID-related GLILD, by assessing symptoms and quality of life alterations, immunological parameters, pulmonary function tests, and lung computed tomography. All six GLILD patients received Rituximab infusions as a first-line treatment. Rituximab was administered at 375 mg/m(2) monthly for six infusions followed by maintenance every 3 months; none of the patients experienced severe adverse events. Symptom burden and quality of life significantly improved in treated patients compared to a control group of CVID patients without GLILD. Rituximab treatment indirectly caused a trend toward reduced T-cell activation and exhaustion markers sCD25 and sTIM-3. Lung function improved in treated patients, with statistically significant increases in TLC and DL(CO). Lung CT scan findings expressed by means of Baumann scoring system displayed a reduction in the entire cohort. In conclusion, first-line monotherapy with Rituximab displayed high efficacy in disease remission in all treated patients, with improvement of symptoms and amelioration of quality of life, as well as restoration of PFTs and lung CT scan findings. SUPPLEMENTARY INFORMATION: The online version contains supplementary material available at 10.1007/s10875-023-01587-4. Springer US 2023-09-27 2023 /pmc/articles/PMC10661825/ /pubmed/37755605 http://dx.doi.org/10.1007/s10875-023-01587-4 Text en © The Author(s) 2023 https://creativecommons.org/licenses/by/4.0/Open Access This article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/ (https://creativecommons.org/licenses/by/4.0/) . |
spellingShingle | Original Article Tessarin, Giulio Baronio, Manuela Gazzurelli, Luisa Rossi, Stefano Chiarini, Marco Moratto, Daniele Giliani, Silvia Clara Bondioni, Maria Pia Badolato, Raffaele Lougaris, Vassilios Rituximab Monotherapy Is Effective as First-Line Treatment for Granulomatous Lymphocytic Interstitial Lung Disease (GLILD) in CVID Patients |
title | Rituximab Monotherapy Is Effective as First-Line Treatment for Granulomatous Lymphocytic Interstitial Lung Disease (GLILD) in CVID Patients |
title_full | Rituximab Monotherapy Is Effective as First-Line Treatment for Granulomatous Lymphocytic Interstitial Lung Disease (GLILD) in CVID Patients |
title_fullStr | Rituximab Monotherapy Is Effective as First-Line Treatment for Granulomatous Lymphocytic Interstitial Lung Disease (GLILD) in CVID Patients |
title_full_unstemmed | Rituximab Monotherapy Is Effective as First-Line Treatment for Granulomatous Lymphocytic Interstitial Lung Disease (GLILD) in CVID Patients |
title_short | Rituximab Monotherapy Is Effective as First-Line Treatment for Granulomatous Lymphocytic Interstitial Lung Disease (GLILD) in CVID Patients |
title_sort | rituximab monotherapy is effective as first-line treatment for granulomatous lymphocytic interstitial lung disease (glild) in cvid patients |
topic | Original Article |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10661825/ https://www.ncbi.nlm.nih.gov/pubmed/37755605 http://dx.doi.org/10.1007/s10875-023-01587-4 |
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