{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,25]],"date-time":"2026-09-25T20:26:43Z","timestamp":1790368003420,"version":"4.1.0"},"reference-count":18,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2018,4,17]],"date-time":"2018-04-17T00:00:00Z","timestamp":1523923200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/501100004587","name":"Instituto de Salud Carlos III","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100004587","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100008530","name":"European Regional Development Fund","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100008530","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Pediatric Allergy Immunology"],"published-print":{"date-parts":[[2018,6]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>One of the most frequent non\u2010infectious complications of humoral immunodeficiencies with a <jats:styled-content style=\"fixed-case\">CVID<\/jats:styled-content>\u2010like pattern is a particular form of inflammatory lung disease which is called granulomatous\u2010lymphocytic interstitial lung disease (<jats:styled-content style=\"fixed-case\">GLILD<\/jats:styled-content>). Its development worsens patient prognosis, with a significant decrease in survival. Currently, there are no unified guidelines regarding its management, and different combinations of immunosuppressants have been used with variable success.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Clinical and radiological data were collected from patient's medical charts. Flow cytometry was performed to characterize the immunological features with special focus in regulatory T cells (Tregs).<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>A 16\u2010year\u2010old girl with Kabuki syndrome and a 12\u2010year\u2010old boy, both with a <jats:styled-content style=\"fixed-case\">CVID<\/jats:styled-content>\u2010like humoral immunodeficiency on immunoglobulin replacement treatment, developed during follow\u2010up an inflammatory complication radiologically, clinically, and histologically compatible with <jats:styled-content style=\"fixed-case\">GLILD<\/jats:styled-content>. They required treatment, and sirolimus was started, with very good response and no serious side effects.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>These 2 cases provide insight into the underlying local and systemic immune anomalies involved in the development of <jats:styled-content style=\"fixed-case\">GLILD<\/jats:styled-content>, including the possible role of Tregs. Combined chemotherapy is commonly used as treatment for <jats:styled-content style=\"fixed-case\">GLILD<\/jats:styled-content> when steroids fail, but there have been some reports of successful monotherapy. As far as we know, these are the first 2 <jats:styled-content style=\"fixed-case\">GLILD<\/jats:styled-content> patients treated successfully with sirolimus, suggesting the advisability of further study of <jats:styled-content style=\"fixed-case\">mTOR<\/jats:styled-content> inhibitors as a more targeted treatment for <jats:styled-content style=\"fixed-case\">GLILD<\/jats:styled-content>, if impairment in Tregs is demonstrated.<\/jats:p><\/jats:sec>","DOI":"10.1111\/pai.12890","type":"journal-article","created":{"date-parts":[[2018,3,15]],"date-time":"2018-03-15T10:14:43Z","timestamp":1521108883000},"page":"425-432","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":39,"title":["Sirolimus as an alternative treatment in patients with granulomatous\u2010lymphocytic lung disease and humoral immunodeficiency with impaired regulatory T cells"],"prefix":"10.1111","volume":"29","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-8026-7674","authenticated-orcid":false,"given":"Angela","family":"Dey\u00e0\u2010Mart\u00ednez","sequence":"first","affiliation":[{"name":"Allergy and Clinical Immunology Department Institut de Recerca Pedi\u00e0trica Hospital Sant Joan de D\u00e9u  Esplugues de Llobregat Spain"},{"name":"Functional Unit of Clinical Immunology Hospital Sant Joan de D\u00e9u\u2010Hospital Clinic Universitat de Barcelona  Barcelona Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0542-0587","authenticated-orcid":false,"given":"Ana","family":"Esteve\u2010Sol\u00e9","sequence":"additional","affiliation":[{"name":"Allergy and Clinical Immunology Department Institut de Recerca Pedi\u00e0trica Hospital Sant Joan de D\u00e9u  Esplugues de Llobregat Spain"},{"name":"Functional Unit of Clinical Immunology Hospital Sant Joan de D\u00e9u\u2010Hospital Clinic Universitat de Barcelona  Barcelona Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Natalia","family":"V\u00e9lez\u2010Tirado","sequence":"additional","affiliation":[{"name":"Universidad Militar Nueva Granada  Bogot\u00e1 Colombia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Veronica","family":"Celis","sequence":"additional","affiliation":[{"name":"Department of Pediatric oncology Institut de Recerca Pedi\u00e0trica Hospital Sant Joan de D\u00e9u  Esplugues de Llobregat Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jordi","family":"Costa","sequence":"additional","affiliation":[{"name":"Department of Pediatric Pneumology Institut de Recerca Pedi\u00e0trica Hospital Sant Joan de D\u00e9u  Esplugues de Llobregat Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maria","family":"Cols","sequence":"additional","affiliation":[{"name":"Department of Pediatric Pneumology Institut de Recerca Pedi\u00e0trica Hospital Sant Joan de D\u00e9u  Esplugues de Llobregat Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Cristina","family":"Jou","sequence":"additional","affiliation":[{"name":"Department of Pathology Hospital Sant Joan de D\u00e9u  Barcelona Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alexandru","family":"Vlagea","sequence":"additional","affiliation":[{"name":"Functional Unit of Clinical Immunology Hospital Sant Joan de D\u00e9u\u2010Hospital Clinic Universitat de Barcelona  Barcelona Spain"},{"name":"Immunology Department Centre de Diagn\u00f2stic Biom\u00e8dic Hospital Cl\u00ednic de Barcelona IDIBAPS Universitat de Barcelona  Barcelona Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ana Mar\u00eda","family":"Plaza\u2010Martin","sequence":"additional","affiliation":[{"name":"Allergy and Clinical Immunology Department Institut de Recerca Pedi\u00e0trica Hospital Sant Joan de D\u00e9u  Esplugues de Llobregat 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