Granulomatous-Lymphocytic Interstitial Lung Disease in Common Variable Immunodeficiency with a Rapid Progression and Refractory Infections.

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.