Related Experiment Video
Updated: Jun 2, 2026

Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
Granulomatous-Lymphocytic Interstitial Lung Disease in Common Variable Immunodeficiency with a Rapid Progression and
Ryuki Tokunaga1, Aiko Masunaga1, Masashi Koga1
1Department of Respiratory Medicine, Kumamoto University Hospital, Japan.
Abstract:
A 34-year-old woman with common variable immunodeficiency (CVID) developed pulmonary abnormalities despite adequate subcutaneous immunoglobulin replacement and long-term low-dose erythromycin therapy. Her lung lesions and hypoxemia progressed, and a surgical biopsy confirmed granulomatous-lymphocytic interstitial lung disease (GLILD). Prednisolone and cyclosporine were administered. However, her condition worsened, and she died of infectious complications. Although patients with CVID-GLILD often respond to immunoglobulin replacement therapy or immunosuppression, this case was rapidly progressive and refractory to treatment.
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