CTICasesGranulomatous-Lymphocytic Interstitial Lung Disease in CVID
Imaging findings
A patient with common variable immunodeficiency has worsening fibrosis with prominent smooth and nodular interlobular septal thickening and hard-looking nodules, with splenomegaly and considerable lymphadenopathy giving a sarcoid-like appearance but with a basal-predominant interstitial distribution. Serial imaging over about five years shows progression from a more nodular pattern in the lower lobes extending superiorly and becoming more fibrotic. Biopsy confirmed granulomatous-lymphocytic interstitial lung disease (GLILD).
Key takeaways
GLILD in common variable immunodeficiency can mimic sarcoidosis with nodular septal thickening and lymphadenopathy but is typically basal-predominant, and biopsy may be dominated by non-necrotizing granulomas or by a lymphocytic (LIP/follicular bronchiolitis-like) component. Recognizing the association with CVID, splenomegaly, and progressive basal fibrosis over years supports the diagnosis of this immune-dysregulation interstitial lung disease.
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