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Evidence-based diagnostic guidelines are lacking, and GLILD treatment consists in immunosuppressive drugs; nonetheless, therapeutical strategies are heterogeneous and essentially based on experts\u2019 opinions and data from small case series or case reports.<\/jats:p>\n                  <jats:p>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.<\/jats:p>\n                  <jats:p>\n                    All six GLILD patients received Rituximab infusions as a first-line treatment. Rituximab was administered at 375 mg\/m\n                    <jats:sup>2<\/jats:sup>\n                    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\n                    <jats:sub>CO<\/jats:sub>\n                    . Lung CT scan findings expressed by means of Baumann scoring system displayed a reduction in the entire cohort.\n                  <\/jats:p>\n                  <jats:p>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.<\/jats:p>","DOI":"10.1007\/s10875-023-01587-4","type":"journal-article","created":{"date-parts":[[2023,9,27]],"date-time":"2023-09-27T11:02:10Z","timestamp":1695812530000},"page":"2091-2103","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":24,"title":["Rituximab Monotherapy Is Effective as First-Line Treatment for Granulomatous Lymphocytic Interstitial Lung Disease (GLILD) in CVID Patients"],"prefix":"10.1007","volume":"43","author":[{"given":"Giulio","family":"Tessarin","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Manuela","family":"Baronio","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Luisa","family":"Gazzurelli","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Stefano","family":"Rossi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marco","family":"Chiarini","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Daniele","family":"Moratto","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Silvia Clara","family":"Giliani","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maria Pia","family":"Bondioni","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Raffaele","family":"Badolato","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2303-9533","authenticated-orcid":false,"given":"Vassilios","family":"Lougaris","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2023,9,27]]},"reference":[{"key":"1587_CR1","doi-asserted-by":"publisher","first-page":"1763","DOI":"10.1016\/j.jaip.2019.02.004","volume":"7","author":"MG Seidel","year":"2019","unstructured":"Seidel MG, Kindle G, Gathmann B, Quinti I, Buckland M, van Montfrans J, et al. 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The study has been approved by the ASST Spedali Civili of Brescia Ethical Committee (NP2972).","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Ethics Approval"}},{"value":"Written informed consent was obtained from all patients (or their caregivers in case of pediatric patients) included in this study.","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Consent to Participate"}},{"value":"Not applicable","order":4,"name":"Ethics","group":{"name":"EthicsHeading","label":"Consent for Publication"}},{"value":"The authors declare no competing interests.","order":5,"name":"Ethics","group":{"name":"EthicsHeading","label":"Conflict of Interest"}}]}}