Successful Treatment of Granulomatous-lymphocytic Interstitial Lung Disease in a Patient with CTLA-4 Deficiency

Common variable immunodeficiency (CVID) causes granulomatous-lymphocytic interstitial lung disease (GLILD) and has a poor prognosis. We herein report a case of GLILD in a 49-year-old woman with CTLA-4 deficiency-associated CVID. The patient presented with dyspnea that had worsened over the past two...

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Autores principales: Nishimura, Masashi, Miyata, Jun, Tanigaki, Tomomi, Nomura, Sakika, Serizawa, Yusuke, Igarashi, Syunya, Itou, Koki, Ohno, Tomohiro, Kurata, Yuhei, Kimizuka, Yoshifumi, Fujikura, Yuji, Sekinaka, Yujin, Sekinaka, Kanako, Matsukuma, Susumu, Nonoyama, Shigeaki, Kawana, Akihiko
Formato: Online Artículo Texto
Lenguaje:English
Publicado: The Japanese Society of Internal Medicine 2022
Materias:
Case Report
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10076143/
https://www.ncbi.nlm.nih.gov/pubmed/35945007
http://dx.doi.org/10.2169/internalmedicine.0076-22
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author Nishimura, Masashi
Miyata, Jun
Tanigaki, Tomomi
Nomura, Sakika
Serizawa, Yusuke
Igarashi, Syunya
Itou, Koki
Ohno, Tomohiro
Kurata, Yuhei
Kimizuka, Yoshifumi
Fujikura, Yuji
Sekinaka, Yujin
Sekinaka, Kanako
Matsukuma, Susumu
Nonoyama, Shigeaki
Kawana, Akihiko
author_facet Nishimura, Masashi
Miyata, Jun
Tanigaki, Tomomi
Nomura, Sakika
Serizawa, Yusuke
Igarashi, Syunya
Itou, Koki
Ohno, Tomohiro
Kurata, Yuhei
Kimizuka, Yoshifumi
Fujikura, Yuji
Sekinaka, Yujin
Sekinaka, Kanako
Matsukuma, Susumu
Nonoyama, Shigeaki
Kawana, Akihiko
author_sort Nishimura, Masashi
collection PubMed
description Common variable immunodeficiency (CVID) causes granulomatous-lymphocytic interstitial lung disease (GLILD) and has a poor prognosis. We herein report a case of GLILD in a 49-year-old woman with CTLA-4 deficiency-associated CVID. The patient presented with dyspnea that had worsened over the past two years. A laboratory examination revealed hypoglobulinemia and pancytopenia. Chest computed tomography showed diffuse infiltrative and granular shadows in the bilateral interstitium. A flow cytometric analysis of blood cells and genetic testing confirmed CTLA-4 deficiency. We performed video-assisted thoracoscopic surgery for the pathological diagnosis of GLILD and to exclude infection and malignancy. Corticosteroid treatment successfully improved the condition of the patient.
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spelling pubmed-100761432023-04-06 Successful Treatment of Granulomatous-lymphocytic Interstitial Lung Disease in a Patient with CTLA-4 Deficiency Nishimura, Masashi Miyata, Jun Tanigaki, Tomomi Nomura, Sakika Serizawa, Yusuke Igarashi, Syunya Itou, Koki Ohno, Tomohiro Kurata, Yuhei Kimizuka, Yoshifumi Fujikura, Yuji Sekinaka, Yujin Sekinaka, Kanako Matsukuma, Susumu Nonoyama, Shigeaki Kawana, Akihiko Intern Med Case Report Common variable immunodeficiency (CVID) causes granulomatous-lymphocytic interstitial lung disease (GLILD) and has a poor prognosis. We herein report a case of GLILD in a 49-year-old woman with CTLA-4 deficiency-associated CVID. The patient presented with dyspnea that had worsened over the past two years. A laboratory examination revealed hypoglobulinemia and pancytopenia. Chest computed tomography showed diffuse infiltrative and granular shadows in the bilateral interstitium. A flow cytometric analysis of blood cells and genetic testing confirmed CTLA-4 deficiency. We performed video-assisted thoracoscopic surgery for the pathological diagnosis of GLILD and to exclude infection and malignancy. Corticosteroid treatment successfully improved the condition of the patient. The Japanese Society of Internal Medicine 2022-08-10 2023-03-15 /pmc/articles/PMC10076143/ /pubmed/35945007 http://dx.doi.org/10.2169/internalmedicine.0076-22 Text en Copyright © 2023 by The Japanese Society of Internal Medicine https://creativecommons.org/licenses/by-nc-nd/4.0/The Internal Medicine is an Open Access journal distributed under the Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License. To view the details of this license, please visit (https://creativecommons.org/licenses/by-nc-nd/4.0/).
spellingShingle Case Report
Nishimura, Masashi
Miyata, Jun
Tanigaki, Tomomi
Nomura, Sakika
Serizawa, Yusuke
Igarashi, Syunya
Itou, Koki
Ohno, Tomohiro
Kurata, Yuhei
Kimizuka, Yoshifumi
Fujikura, Yuji
Sekinaka, Yujin
Sekinaka, Kanako
Matsukuma, Susumu
Nonoyama, Shigeaki
Kawana, Akihiko
Successful Treatment of Granulomatous-lymphocytic Interstitial Lung Disease in a Patient with CTLA-4 Deficiency
title Successful Treatment of Granulomatous-lymphocytic Interstitial Lung Disease in a Patient with CTLA-4 Deficiency
title_full Successful Treatment of Granulomatous-lymphocytic Interstitial Lung Disease in a Patient with CTLA-4 Deficiency
title_fullStr Successful Treatment of Granulomatous-lymphocytic Interstitial Lung Disease in a Patient with CTLA-4 Deficiency
title_full_unstemmed Successful Treatment of Granulomatous-lymphocytic Interstitial Lung Disease in a Patient with CTLA-4 Deficiency
title_short Successful Treatment of Granulomatous-lymphocytic Interstitial Lung Disease in a Patient with CTLA-4 Deficiency
title_sort successful treatment of granulomatous-lymphocytic interstitial lung disease in a patient with ctla-4 deficiency
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10076143/
https://www.ncbi.nlm.nih.gov/pubmed/35945007
http://dx.doi.org/10.2169/internalmedicine.0076-22
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