Magnetic Resonance Elastography of Liver in Light Chain Amyloidosis.

Sudhakar K Venkatesh,Michael S Torbenson,Richard L Ehman,Safa Hoodeshenas,Angela Dispenzieri,Morie A Gertz,Sandeep H Venkatesh

doi:10.3390/jcm8050739

Sudhakar K Venkatesh, Michael S Torbenson + Show 5 more

Open Access

https://doi.org/10.3390/jcm8050739

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Abstract

In this paper, we present our preliminary findings regarding magnetic resonance elastography (MRE) on the livers of 10 patients with systemic amyloidosis. Mean liver stiffness measurements (LSM) and spleen stiffness measurements (SSM) were obtained. Magnetic resonance imaging (MRI) images were analyzed for the distribution pattern of amyloid deposition. Pearson correlation analysis was performed in order to study the correlation between LSM, SSM, liver span, liver volume, spleen span, spleen volume, serum alkaline phosphatase (ALP), N-terminal pro b-type natriuretic peptide (NT pro BNP), and the kappa and lambda free light chains. An increase in mean LSM was seen in all patients. Pearson correlation analysis showed a statistically significant correlation between LSM and liver volume (r = 0.78, p = 0.007) and kappa chain level (r = 0.65, p = 0.04). Interestingly, LSM did not correlate significantly with SSM (r = 0.45, p = 0.18), liver span (r = 0.57, p = 0.08), or serum ALP (r = 0.60, p = 0.07). However, LSM correlated significantly with serum ALP when corrected for liver volume (partial correlation, r = 0.71, p = 0.03) and NT pro BNP levels (partial correlation, r = 0.68, p = 0.04). MRI review revealed that amyloid deposition in the liver can be diffuse, lobar, or focal. MRE is useful for the evaluation of hepatic amyloidosis and shows increased stiffness in hepatic amyloidosis. MRE has the potential to be a non-invasive quantitative imaging marker for hepatic amyloidosis.

Highlights

Hepatic involvement in systemic amyloidosis is common
Hepatic amyloidosis (HA) is characterized by the deposition of amyloid fibrils in the space of Disse that usually begins in the periportal region, occasionally it is centrilobular and may be deposited in the hepatic vasculature [4,6]
Amyloid deposition leads to pressure atrophy of the hepatocytes, which interferes with the passage of bile resulting in cholestasis, or it may block the sinusoids, leading to portal hypertension [4,6,7,8]

Summary

Introduction

Hepatic involvement in systemic amyloidosis is common. It is found in 22%–95% of patients with a higher prevalence of primary amyloidosis [1,2,3,4,5]. Liver involvement and response assessment is performed by measuring liver span on imaging modalities and correlating this with serum alkaline phosphatase levels. MRE may be useful in the evaluation of HA given its proven value in hepatic fibrosis, as amyloidosis is an extracellular process and associated with increased stiffness.

Results

Conclusion