Interstitial lung disease associated with immunodeficiency with hypogammaglobulinemia: Five case reports

Immunodeficiency with hypogammaglobulinemia is associated with interstitial lung disease, but its mechanism and treatment remain unclear and may differ from ordinary interstitial pneumonia. Herein, we report five such cases. Lung biopsy showed a benign lymphoid infiltration pattern and granulomatous lymphocytic interstitial lung disease. Treatment with corticosteroids and immunosuppressants was usually effective, but it was ineffective in these cases. All cases treated with intravenous immunoglobulin showed improvement. In interstitial lung disease that is unresponsive to conventional treatment, including corticosteroids and immunosuppressants, hypogammaglobulinemia should be suspected.