Diagnostic performance of liver stiffness as marker of liver involvement in systemic immunoglobulin light chain (AL) amyloidosis

Anne F Brunger; Hendrea S A Tingen; Johan Bijzet; Ronald van Rheenen; Hans Blokzijl; Wilfried W H Roeloffzen; Ewout J Houwerzijl; Friso L H Muntinghe; Riemer H J A Slart; Reinold O B Gans; Christoph Kimmich; Bouke P C Hazenberg; Hans L A Nienhuis

doi:10.1007/s00277-024-05932-4

Diagnostic performance of liver stiffness as marker of liver involvement in systemic immunoglobulin light chain (AL) amyloidosis

Anne F Brunger^*, Hendrea S A Tingen, Johan Bijzet, Ronald van Rheenen, Hans Blokzijl, Wilfried W H Roeloffzen, Ewout J Houwerzijl, Friso L H Muntinghe, Riemer H J A Slart, Reinold O B Gans, Christoph Kimmich, Bouke P C Hazenberg, Hans L A Nienhuis

^*Corresponding author voor dit werk

Onderzoeksoutput › Academic › peer review

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OBJECTIVE: To investigate the diagnostic performance of liver stiffness for detecting liver involvement in immunoglobulin light chain (AL) amyloidosis.

METHODS: Liver stiffness was measured using transient elastography in 71 patients with systemic AL amyloidosis and 18 patients with wild type transthyretin (ATTRwt) amyloidosis with cardiomyopathy. Both non-invasive consensus criteria and serum amyloid P component (SAP) scintigraphy were used as substitute standards instead of liver biopsy for establishing liver involvement.

RESULTS: Liver stiffness was higher in AL amyloidosis patients with liver involvement than in those without: this was observed using both consensus criteria (median 14.4 kPa vs. 8.1 kPa; p = 0.001) and SAP scintigraphy (median 20.9 kPa vs. 6.2 kPa; p < 0.001). Liver stiffness was also higher in AL amyloidosis patients with liver involvement compared to AL and ATTRwt amyloidosis patients with cardiac involvement. Based on receiver operating characteristic (ROC) curves a cut-off value of 14.4 kPa for stiffness was optimal to indicate liver involvement, providing sensitivity and specificity of 50% and 74%, respectively, using the consensus criteria and 63% and 90%, respectively, using SAP scintigraphy as standard.

CONCLUSION: Liver stiffness is a promising tool to establish liver involvement in AL amyloidosis having potential to become part of updated criteria for liver involvement.

Originele taal-2	English
Tijdschrift	Annals of Hematology
DOI's	https://doi.org/10.1007/s00277-024-05932-4
Status	E-pub ahead of print - 16-aug.-2024

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Diagnostic performance of liver stiffness as marker of liver involvement in systemic immunoglobulin light chain (AL) amyloidosisFinal publisher's version, 1,18 MBLicentie: CC BY

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Brunger, A. F., Tingen, H. S. A., Bijzet, J., van Rheenen, R., Blokzijl, H., Roeloffzen, W. W. H., Houwerzijl, E. J., Muntinghe, F. L. H., Slart, R. H. J. A., Gans, R. O. B., Kimmich, C., Hazenberg, B. P. C., & Nienhuis, H. L. A. (2024). Diagnostic performance of liver stiffness as marker of liver involvement in systemic immunoglobulin light chain (AL) amyloidosis. Annals of Hematology. Online publicatie vooraf. https://doi.org/10.1007/s00277-024-05932-4

@article{eff692227b5c4e72978cf1ac8e296f5d,

title = "Diagnostic performance of liver stiffness as marker of liver involvement in systemic immunoglobulin light chain (AL) amyloidosis",

abstract = "OBJECTIVE: To investigate the diagnostic performance of liver stiffness for detecting liver involvement in immunoglobulin light chain (AL) amyloidosis.METHODS: Liver stiffness was measured using transient elastography in 71 patients with systemic AL amyloidosis and 18 patients with wild type transthyretin (ATTRwt) amyloidosis with cardiomyopathy. Both non-invasive consensus criteria and serum amyloid P component (SAP) scintigraphy were used as substitute standards instead of liver biopsy for establishing liver involvement.RESULTS: Liver stiffness was higher in AL amyloidosis patients with liver involvement than in those without: this was observed using both consensus criteria (median 14.4 kPa vs. 8.1 kPa; p = 0.001) and SAP scintigraphy (median 20.9 kPa vs. 6.2 kPa; p < 0.001). Liver stiffness was also higher in AL amyloidosis patients with liver involvement compared to AL and ATTRwt amyloidosis patients with cardiac involvement. Based on receiver operating characteristic (ROC) curves a cut-off value of 14.4 kPa for stiffness was optimal to indicate liver involvement, providing sensitivity and specificity of 50% and 74%, respectively, using the consensus criteria and 63% and 90%, respectively, using SAP scintigraphy as standard.CONCLUSION: Liver stiffness is a promising tool to establish liver involvement in AL amyloidosis having potential to become part of updated criteria for liver involvement.",

author = "Brunger, {Anne F} and Tingen, {Hendrea S A} and Johan Bijzet and {van Rheenen}, Ronald and Hans Blokzijl and Roeloffzen, {Wilfried W H} and Houwerzijl, {Ewout J} and Muntinghe, {Friso L H} and Slart, {Riemer H J A} and Gans, {Reinold O B} and Christoph Kimmich and Hazenberg, {Bouke P C} and Nienhuis, {Hans L A}",

note = "{\textcopyright} 2024. The Author(s).",

year = "2024",

month = aug,

day = "16",

doi = "10.1007/s00277-024-05932-4",

language = "English",

journal = "Annals of Hematology",

issn = "0939-5555",

publisher = "Springer",

}

Brunger, AF , Tingen, HSA , Bijzet, J, van Rheenen, R, Blokzijl, H , Roeloffzen, WWH, Houwerzijl, EJ, Muntinghe, FLH , Slart, RHJA , Gans, ROB, Kimmich, C, Hazenberg, BPC & Nienhuis, HLA 2024, 'Diagnostic performance of liver stiffness as marker of liver involvement in systemic immunoglobulin light chain (AL) amyloidosis', Annals of Hematology. https://doi.org/10.1007/s00277-024-05932-4

TY - JOUR

T1 - Diagnostic performance of liver stiffness as marker of liver involvement in systemic immunoglobulin light chain (AL) amyloidosis

AU - Brunger, Anne F

AU - Tingen, Hendrea S A

AU - Bijzet, Johan

AU - van Rheenen, Ronald

AU - Blokzijl, Hans

AU - Roeloffzen, Wilfried W H

AU - Houwerzijl, Ewout J

AU - Muntinghe, Friso L H

AU - Slart, Riemer H J A

AU - Gans, Reinold O B

AU - Kimmich, Christoph

AU - Hazenberg, Bouke P C

AU - Nienhuis, Hans L A

PY - 2024/8/16

Y1 - 2024/8/16

N2 - OBJECTIVE: To investigate the diagnostic performance of liver stiffness for detecting liver involvement in immunoglobulin light chain (AL) amyloidosis.METHODS: Liver stiffness was measured using transient elastography in 71 patients with systemic AL amyloidosis and 18 patients with wild type transthyretin (ATTRwt) amyloidosis with cardiomyopathy. Both non-invasive consensus criteria and serum amyloid P component (SAP) scintigraphy were used as substitute standards instead of liver biopsy for establishing liver involvement.RESULTS: Liver stiffness was higher in AL amyloidosis patients with liver involvement than in those without: this was observed using both consensus criteria (median 14.4 kPa vs. 8.1 kPa; p = 0.001) and SAP scintigraphy (median 20.9 kPa vs. 6.2 kPa; p < 0.001). Liver stiffness was also higher in AL amyloidosis patients with liver involvement compared to AL and ATTRwt amyloidosis patients with cardiac involvement. Based on receiver operating characteristic (ROC) curves a cut-off value of 14.4 kPa for stiffness was optimal to indicate liver involvement, providing sensitivity and specificity of 50% and 74%, respectively, using the consensus criteria and 63% and 90%, respectively, using SAP scintigraphy as standard.CONCLUSION: Liver stiffness is a promising tool to establish liver involvement in AL amyloidosis having potential to become part of updated criteria for liver involvement.

AB - OBJECTIVE: To investigate the diagnostic performance of liver stiffness for detecting liver involvement in immunoglobulin light chain (AL) amyloidosis.METHODS: Liver stiffness was measured using transient elastography in 71 patients with systemic AL amyloidosis and 18 patients with wild type transthyretin (ATTRwt) amyloidosis with cardiomyopathy. Both non-invasive consensus criteria and serum amyloid P component (SAP) scintigraphy were used as substitute standards instead of liver biopsy for establishing liver involvement.RESULTS: Liver stiffness was higher in AL amyloidosis patients with liver involvement than in those without: this was observed using both consensus criteria (median 14.4 kPa vs. 8.1 kPa; p = 0.001) and SAP scintigraphy (median 20.9 kPa vs. 6.2 kPa; p < 0.001). Liver stiffness was also higher in AL amyloidosis patients with liver involvement compared to AL and ATTRwt amyloidosis patients with cardiac involvement. Based on receiver operating characteristic (ROC) curves a cut-off value of 14.4 kPa for stiffness was optimal to indicate liver involvement, providing sensitivity and specificity of 50% and 74%, respectively, using the consensus criteria and 63% and 90%, respectively, using SAP scintigraphy as standard.CONCLUSION: Liver stiffness is a promising tool to establish liver involvement in AL amyloidosis having potential to become part of updated criteria for liver involvement.

U2 - 10.1007/s00277-024-05932-4

DO - 10.1007/s00277-024-05932-4

M3 - Article

C2 - 39147985

SN - 0939-5555

JO - Annals of Hematology

JF - Annals of Hematology

ER -

Diagnostic performance of liver stiffness as marker of liver involvement in systemic immunoglobulin light chain (AL) amyloidosis

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