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 journalArticlepeer-review

1 Citation (Scopus)

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 languageEnglish
Pages (from-to)871-875
Number of pages5
JournalInternal Medicine
Volume62
Issue number6
DOIs
Publication statusPublished - 2023
Externally publishedYes

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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