Serum amyloid P component scintigraphy in familial amyloid polyneuropathy: regression of visceral amyloid following liver transplantation.
Familial amyloid polyneuropathy (FAP) associated with transthyretin (TTR) mutations is the commonest type of hereditary amyloidosis. Plasma TTR is produced almost exclusively in the liver and orthotopic liver transplantation is the only available treatment, although the clinical outcome varies. Serum amyloid P component (SAP) scintigraphy is a method for identifying and quantitatively monitoring amyloid deposits in vivo, but it has not previously been used to study the outcome of visceral amyloid deposits in FAP following liver transplantation. Whole body scintigraphy following injection of iodine-123 labelled SAP was performed in 17 patients with FAP associated with TTR Met30 and in five asymptomatic gene carriers. Follow-up studies were performed in ten patients, eight of whom had undergone orthotopic liver transplantation 1-5 years beforehand. There was abnormal uptake of 123I-SAP in all FAP patients, including the kidneys in each case, the spleen in five cases and the adrenal glands in three cases. Renal amyloid deposits were also present in three of the asymptomatic carriers. Follow-up studies 1-5 years after liver transplantation showed that there had been substantial regression of the visceral amyloid deposits in two patients and modest improvement in three cases. The amyloid deposits were unchanged in two patients. In conclusion, 123I-SAP scintigraphy identified unsuspected visceral amyloid in each patient with FAP due to TTR Met30. The universal presence of renal amyloid probably underlies the high frequency of renal failure that occurs in FAP following liver transplantation. The variable capacity of patients to mobilise amyloid deposits following liver transplantation may contribute to their long-term clinical outcome.
Rydh
A
A
Department of Diagnostic Radiology, University Hospital of Northern Sweden, Umeå, Sweden.
Suhr
O
O
Hietala
S O
SO
Ahlström
K R
KR
Pepys
M B
MB
Hawkins
P N
PN
D000328:Adult; D017772:Amyloid Neuropathies / Q000000981:diagnostic imaging* / Q000235:genetics / Q000601:surgery; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D016031:Liver Transplantation / Q000000981:diagnostic imaging*; D008297:Male; D008875:Middle Aged; D011877:Radionuclide Imaging; D000683:Serum Amyloid P-Component / Q000378:metabolism*; D016896:Treatment Outcome
D000683:Serum Amyloid P-Component