scholarly journals Diagnostic Performance and Prognostic Value of Extravascular Retention of 123I-Labeled Serum Amyloid P Component in Systemic Amyloidosis

2007 ◽  
Vol 48 (6) ◽  
pp. 865-872 ◽  
Author(s):  
B. P.C. Hazenberg ◽  
M. H. van Rijswijk ◽  
M. N. Lub-de Hooge ◽  
E. Vellenga ◽  
E. B. Haagsma ◽  
...  
Gerontology ◽  
1991 ◽  
Vol 37 (1) ◽  
pp. 56-62 ◽  
Author(s):  
F. Tashiro ◽  
S. Yi ◽  
S. Wakasugi ◽  
S. Maeda ◽  
K. Shimada ◽  
...  

2006 ◽  
Vol 119 (4) ◽  
pp. 355.e15-355.e24 ◽  
Author(s):  
Bouke P.C. Hazenberg ◽  
Martin H. van Rijswijk ◽  
D. Albertus Piers ◽  
Marjolijn N. Lub-de Hooge ◽  
Edo Vellenga ◽  
...  

2018 ◽  
Vol 10 (422) ◽  
pp. eaan3128 ◽  
Author(s):  
Duncan B. Richards ◽  
Louise M. Cookson ◽  
Sharon V. Barton ◽  
Lia Liefaard ◽  
Thirusha Lane ◽  
...  

Systemic amyloidosis is a fatal disorder caused by pathological extracellular deposits of amyloid fibrils that are always coated with the normal plasma protein, serum amyloid P component (SAP). The small-molecule drug, miridesap, [(R)-1-[6-[(R)-2-carboxy-pyrrolidin-1-yl]-6-oxo-hexanoyl]pyrrolidine-2-carboxylic acid (CPHPC)] depletes circulating SAP but leaves some SAP in amyloid deposits. This residual SAP is a specific target for dezamizumab, a fully humanized monoclonal IgG1 anti-SAP antibody that triggers immunotherapeutic clearance of amyloid. We report the safety, pharmacokinetics, and dose-response effects of up to three cycles of miridesap followed by dezamizumab in 23 adult subjects with systemic amyloidosis (ClinicalTrials.gov identifier:NCT01777243). Amyloid load was measured scintigraphically by amyloid-specific radioligand binding of123I-labeled SAP or of99mTc-3,3-diphosphono-1,2-propanodicarboxylic acid. Organ extracellular volume was measured by equilibrium magnetic resonance imaging and liver stiffness by transient elastography. The treatment was well tolerated with the main adverse event being self-limiting early onset rashes after higher antibody doses related to whole body amyloid load. Progressive dose-related clearance of hepatic amyloid was associated with improved liver function tests.123I-SAP scintigraphy confirmed amyloid removal from the spleen and kidneys. No adverse cardiac events attributable to the intervention occurred in the six subjects with cardiac amyloidosis. Amyloid load reduction by miridesap treatment followed by dezamizumab has the potential to improve management and outcome in systemic amyloidosis.


Gut ◽  
1998 ◽  
Vol 42 (5) ◽  
pp. 727-734 ◽  
Author(s):  
L B Lovat ◽  
M R Persey ◽  
S Madhoo ◽  
M B Pepys ◽  
P N Hawkins

Background and aims—The liver is frequently involved in amyloidosis but the significance of hepatic amyloid has not been systematically studied. We have previously developed scintigraphy with 123I serum amyloid P component (123I-SAP) to identify and monitor amyloid deposits quantitatively in vivo and we report here our findings in hepatic amyloidosis.Methods—Between 1988 and 1995, 805 patients with clinically suspected or biopsy proven systemic amyloidosis were evaluated. One hundred and thirty eight patients had AA amyloidosis, 180 had AL amyloidosis, 99 had hereditary amyloid syndromes, and 67 had dialysis related (β2 microglobulin) amyloid. One hundred and ninety two patients with amyloidosis were followed for six months to eight years.Results—Hepatic amyloid was found in 98/180 (54%) AL and 25/138 (18%) AA patients but in only 1/53 patients with familial transthyretin amyloid polyneuropathy and in none with dialysis related amyloidosis. There was complete concordance between hepatic SAP scintigraphy and the presence or absence of parenchymal amyloid deposits on liver histology. Amyloidosis was never confined to the liver. Mortality was rarely due to hepatic failure, although hepatic involvement with AA amyloid carried a poor prognosis. Successful therapy to reduce the supply of amyloid fibril protein precursors was followed by substantial regression of all types of amyloid.Conclusions—SAP scintigraphy is a specific and sensitive method for detecting and monitoring hepatic amyloid. Liver involvement is always associated with major amyloid in other organ systems and carries a poor prognosis in AA type. Appropriate therapy may substantially improve prognosis in many patients.


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