Non-invasive assessment of hepatic involvement in common variable immunodeficiency and agammaglobulinemia using enhanced liver fibrosis score and shearwave elastography.

BACKGROUND

Early detection of hepatic involvement in common variable immunodeficiency (CVID) and agammaglobulinemia (AGA) remains hampered by the absence of validated non-invasive biomarkers. Although the Enhanced Liver Fibrosis (ELF) score is well established across different liver diseases, its role in CVID/AGA remains undefined. We aimed to assess hepatic involvement using ELF and shearwave elastography (SWE), and to evaluate their correlation and agreement.

METHODS

This cross-sectional study included 22 patients with CVID and 9 with AGA without previously known/documented liver fibrosis. The ELF score was calculated from serum hyaluronic acid (HA), tissue inhibitor of metalloproteinases-1, and N-terminal propeptide of type III collagen. Liver stiffness (LS) was measured using SWE. False discovery rate was controlled using the Benjamini-Hochberg method.

RESULTS

According to SWE, 64.5% of patients had elevated LS, whereas increased ELF values corresponding to intermediate and severe/advanced fibrosis were present in 58% and 35.5%, respectively. There was a statistically significant agreement (weighted Cohen's κ=0.324; p=0.002) along with a significant correlation between ELF and LS (p=0.01/pbh=0.05). In addition, ELF values were also positively associated with AST (p<0.001/pbh<0.001) and ALP (p=0.003/pbh=0.03). Among individual ELF components, HA showed consistent associations with LS and liver enzymes, including ALT, AST, and γGT (p=0.01/pbh=0.05; p<0.001/pbh<0.001; p=0.004/pbh=0.04, respectively).

CONCLUSION

Our exploratory findings suggest that combining ELF and SWE may provide a promising non-invasive approach for liver assessment, potentially reflecting different but complementary aspects of hepatic involvement in CVID and AGA; however, their clinical utility requires validation in larger, prospective, multicenter studies with histopathological correlation to define disease-specific thresholds.