Patients with common variable immunodeficiency (CVID) frequently present raised serum levels of liver enzymes and/or mild hepatomegaly. Fibroscan® is an ultrasound-based transient elastography (TE) method that is widely employed for monitoring liver stiffness in chronic liver diseases but has never been used to evaluate liver involvement in CVID. We performed a cross-sectional study to assess liver involvement in a cohort of adult CVID-patients by the use of Fibroscan.
Haematological exams, liver enzymes, hepato-splenic ultrasounds and Fibroscan® were performed in 77 adult CVID patients. Immunological and clinical features of patients were recorded from medical charts (Table 1).
Study Population (n = 77) | |
Clinical phenotype*, n Infections only Autoimmune cytopenias Polyclonal lymphoproliferation Enteropathy Malignancies | 20 16 36 28 6 |
Fibrosis score, n (%) F0/F1: no to mild fibrosis F2: moderate fibrosis F3: severe fibrosis F4: cirrhosis | 51 18 6 2 |
33.8% (26/77) patients had elevated liver stiffness values ranging from moderate fibrosis to cirrhosis. Fibroscan® values were linearly correlated with ALP, γGT, spleen longitudinal diameter and peripheral blood counts (no correlation with BMI, AST, ALT, total proteins, albumin, bilirubin and hemoglobin was observed) (Fig. 1). Fibroscan® values were higher in subjects with polyclonal lymphoproliferation and enteropathy (Fig. 2), and patients with both these manifestations were more likely to present pathologic TE values (OR: 7.14) compared with those without anyone of these phenotypes (Fig. 3).
Fibroscan® is a pivotal tool to be used in association with clinical and laboratory data to estimate liver disease and guide therapeutic interventions in CVID.