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

Masashi Nishimura; Jun Miyata; Tomomi Tanigaki; Sakika Nomura; Yusuke Serizawa; Syunya Igarashi; Koki Itou; Tomohiro Ohno; Yuhei Kurata; Yoshifumi Kimizuka; Yuji Fujikura; Yujin Sekinaka; Kanako Sekinaka; Susumu Matsukuma; Shigeaki Nonoyama; Akihiko Kawana

doi:10.2169/internalmedicine.0076-22

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

Masashi Nishimura, Jun Miyata, Tomomi Tanigaki, Sakika Nomura, Yusuke Serizawa, Syunya Igarashi, Koki Itou, Tomohiro Ohno, Yuhei Kurata, Yoshifumi Kimizuka, Yuji Fujikura, Yujin Sekinaka, Kanako Sekinaka, Susumu Matsukuma, Shigeaki Nonoyama, Akihiko Kawana

Research output: Contribution to journal › Article › peer-review

Abstract

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.

Original language	English
Pages (from-to)	871-875
Number of pages	5
Journal	Internal Medicine
Volume	62
Issue number	6
DOIs	https://doi.org/10.2169/internalmedicine.0076-22
Publication status	Published - 2023
Externally published	Yes

Keywords

CTLA-4 deficiency
common variable immunodeficiency
corticosteroid
granulomatouslymphocytic interstitial lung disease
video-assisted thoracoscopic surgery

ASJC Scopus subject areas

Internal Medicine

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10.2169/internalmedicine.0076-22

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Nishimura, M., Miyata, J., Tanigaki, T., Nomura, S., Serizawa, Y., Igarashi, S., Itou, K., Ohno, T., Kurata, Y., Kimizuka, Y., Fujikura, Y., Sekinaka, Y., Sekinaka, K., Matsukuma, S., Nonoyama, S., & Kawana, A. (2023). Successful Treatment of Granulomatous-lymphocytic Interstitial Lung Disease in a Patient with CTLA-4 Deficiency. Internal Medicine, 62(6), 871-875. https://doi.org/10.2169/internalmedicine.0076-22

Nishimura, M, Miyata, J, Tanigaki, T, Nomura, S, Serizawa, Y, Igarashi, S, Itou, K, Ohno, T, Kurata, Y, Kimizuka, Y, Fujikura, Y, Sekinaka, Y, Sekinaka, K, Matsukuma, S, Nonoyama, S & Kawana, A 2023, 'Successful Treatment of Granulomatous-lymphocytic Interstitial Lung Disease in a Patient with CTLA-4 Deficiency', Internal Medicine, vol. 62, no. 6, pp. 871-875. https://doi.org/10.2169/internalmedicine.0076-22

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

abstract = "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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author = "Masashi Nishimura and Jun Miyata and Tomomi Tanigaki and Sakika Nomura and Yusuke Serizawa and Syunya Igarashi and Koki Itou and Tomohiro Ohno and Yuhei Kurata and Yoshifumi Kimizuka and Yuji Fujikura and Yujin Sekinaka and Kanako Sekinaka and Susumu Matsukuma and Shigeaki Nonoyama and Akihiko Kawana",

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AU - Nishimura, Masashi

AU - Miyata, Jun

AU - Tanigaki, Tomomi

AU - Nomura, Sakika

AU - Serizawa, Yusuke

AU - Igarashi, Syunya

AU - Itou, Koki

AU - Ohno, Tomohiro

AU - Kurata, Yuhei

AU - Kimizuka, Yoshifumi

AU - Fujikura, Yuji

AU - Sekinaka, Yujin

AU - Sekinaka, Kanako

AU - Matsukuma, Susumu

AU - Nonoyama, Shigeaki

AU - Kawana, Akihiko

PY - 2023

Y1 - 2023

N2 - 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.

AB - 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.

KW - CTLA-4 deficiency

KW - common variable immunodeficiency

KW - corticosteroid

KW - granulomatouslymphocytic interstitial lung disease

KW - video-assisted thoracoscopic surgery

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

Abstract

Keywords

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