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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Detalles Bibliográficos
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
Descripción
Sumario: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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