Granulomatous lymphocytic interstitial lung disease

Last revised by Yuranga Weerakkody on 28 Jan 2024

Granulomatous-lymphocytic interstitial lung disease (GL-ILD) is a relatively recent term given to describe non-infectious diffuse lung disease complications that have been reported to traditionally develop in common variable immunodeficiency (CVID) patients.

Although it is almost always associated with CVID, some papers also report the occurrence of these conditions with primary immunodeficiency states such as 6:

They exhibit both granulomatous and lymphoproliferative histologic patterns consisting of:

Pulmonary findings tend to have a mid- to lower zones distribution and are usually better depicted on CT 7, including: 

  • diffuse micronodules 
  • interlobular septal thickening 
  • bronchiectasis: tend to be mild 
  • lymphadenopathy 

Splenomegaly is almost always seen in those patients. 

Immunosuppressive therapy (e.g. rituximab) has been successfully used in the treatment of granulomatous lymphocytic interstitial lung disease. The presence of granulomatous lymphocytic interstitial lung disease in association with common variable immunodeficiency confers a poorer prognosis and also carries an increased risk of lymphoproliferative disorders.

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