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

• Published in: Internal Medicine Vol. 62; no. 6; pp. 871 - 875
• Main Authors: 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
• Format: Journal Article
• Language: English
• Published: Japan The Japanese Society of Internal Medicine 15.03.2023; Japan Science and Technology Agency
• Subjects: Blood cells; Case Report; Case reports; Common variable immunodeficiency; Common Variable Immunodeficiency - complications; Common Variable Immunodeficiency - diagnosis; Common Variable Immunodeficiency - drug therapy; Computed tomography; corticosteroid; CTLA-4 Antigen; CTLA-4 deficiency; CTLA-4 protein; Dyspnea; Female; Flow cytometry; Genetic analysis; Genetic screening; Granuloma - diagnosis; granulomatous-lymphocytic interstitial lung disease; Humans; Internal medicine; Lung diseases; Lung Diseases, Interstitial - complications; Lung Diseases, Interstitial - diagnostic imaging; Lung Diseases, Interstitial - drug therapy; Malignancy; Middle Aged; Pancytopenia; Patients; Respiration; Tomography, X-Ray Computed - adverse effects; video-assisted thoracoscopic surgery; granulomatous-lymphocytic interstitial lung disease; corticosteroid; CTLA-4 deficiency; common variable immunodeficiency; video-assisted thoracoscopic surgery
• ISSN: 0918-2918; 1349-7235; 1349-7235
• DOI: 10.2169/internalmedicine.0076-22

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.