The Utility of Liver Elastography in the Evaluation of Nodular Regenerative Hyperplasia (NRH) in Patients with Common Variable Immune Deficiency (CVID)

Abstract

NRH has been correlated with increased morbidity and mortality in CVID patients. Liver biopsy is the gold standard for NRH diagnosis, however, ideal would be an earlier, noninvasive diagnostic technique. We hypothesized that Fibroscan® (vibration controlled transient liver elastography) may facilitate the diagnosis of NRH in CVID patients. Using a published MGH CVID cohort, we identified CVID patients with biopsy-confirmed NRH (‘NRH’), elevated liver biochemistries without biopsy-confirmed NRH (‘at risk for NRH’), and non-elevated liver biochemistries (‘control’). Patients with chronic viral hepatitis, non-alcoholic steatohepatitis, chronic alcohol use, and/or primary biliary cirrhosis were excluded. We reviewed both clinical Fibroscans, from the electronic medical record, and research-based Fibroscans. Fibroscan-obtained liver stiffness measurements (LSM, kPa) were compared using a two-tailed Student’s t-test. Within the MGH CVID cohort, ‘control’ patients (n=3) had a median LSM of 4.9 kPa (range 4.1 – 5.8 kPa), ‘NRH’ patients (n=4) had a median LSM of 11.2 kPa (range 7.0 – 26.1 kPa), and ‘at risk for NRH’ patients (n=2) had a median LSM of 11.5 kPa (range 9.8-13.1 kPa). Compared to control CVID, LSMs were significantly elevated in CVID patients with known or suspected NRH (P = 0.031). These preliminary data suggest the diagnostic utility of Fibroscan in CVID patients with known or suspected NRH. Elevated liver biochemistries and LSMs may prompt liver biopsy and immediate immunosuppressive intervention. Further studies will extend these findings to the full CVID cohort, correlate Fibroscan reads with NRH disease severity on biopsy, and increase monitoring of liver stiffness following immunosuppressive therapy.