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• W4300407248 abstract "Background Hepatic involvement in AL amyloidosis is often asymptomatic but does affect prognosis and should be taken into account during follow-up. An increased plasma level of alkaline phosphatase (ALP) or increased liver span are part of the conventional diagnostic criteria for establishing hepatic involvement 1 , however these markers are nonspecific. 123 I-labeled serum amyloid P component (SAP) scintigraphy is a specific and sensitive method to establish hepatic involvement but is not widely available. Liver stiffness measured by transient elastography is increased in AL amyloidosis patients with hepatic involvement and could be useful in establishing liver involvement and monitoring treatment response in AL-amyloidosis. Objectives To assess the diagnostic value of liver stiffness (LS) for liver involvement in AL amyloidosis and the utility of LS to monitor treatment effect using SAP scintigraphy as gold standard. Methods LS was measured prospectively in 49 treatment naive patients with systemic AL amyloidosis and 9 patients with wild type transthyretin amyloidosis (ATTRwt) with cardiomyopathy (cardiac controls). In addition, LS was longitudinally measured in 10 AL amyloidosis patients of whom 9 patients had liver involvement. SAP scintigraphy, laboratory assessments including ALP and measurement of liver span was performed in all patients. Results Of the 49 patients, 27 patients had liver involvement (of whom 24 also had heart involvement), 10 patients had heart involvement, 12 patients had no heart or liver involvement. Median LS was significantly higher in AL amyloidosis patients with liver involvement (22.8 kPa, range 4.3-75), than in AL amyloidosis patients without liver involvement (6.3 kPa, range 4.4-35.8) (p=0.000). Also a significant difference was seen between AL amyloidosis patients with liver involvement (22.8.9, range 4.3-75) versus, heart involvement (9.0, range 4.5-35.8) (p=0.02), no liver or heart involvement (5.7, range 4.4-10.1) (p=0.0001), and ATTRwt patients (9.9 kPa, range 4.0-14.6) (p=0.01). Furthermore, LS values seemed to significantly decrease over time in AL amyloidosis patients with liver involvement with a good hematologic response to treatment. Conclusion LS is a non-invasive tool which seems to be useful in clinical practice to establish liver involvement in patients with AL amyloidosis. In addition, it is a promising marker in the follow up of AL amyloidosis patients for establishing hepatic response over time. References [1]Gertz et al. Am J Hematol 2005;79319-328 Disclosure of Interests None declared" @default.
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• W4300407248 date "2022-05-23" @default.
• W4300407248 modified "2023-09-23" @default.
• W4300407248 title "POS0124 DIAGNOSTIC VALUE OF LIVER STIFFNESS AS MARKER OF HEPATIC MARKER OF HEPATIC AMYLOID DEPOSITION IN SYSTEMIC AL AMYLOIDOSIS" @default.
• W4300407248 doi "https://doi.org/10.1136/annrheumdis-2022-eular.4347" @default.
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