The liver in systemic amyloidosis: insights from ¹²³I serum amyloid P component scintigraphy in 484 patients

Abstract

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 123 I serum amyloid P component ( 123 I-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 ( 2 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. (Gut 1998;42:727-734)

Repeat doses of antibody to serum amyloid P component clear amyloid deposits in patients with systemic amyloidosis

“…Serial doses, comprising sufficient dezamizumab in relation to each subject's amyloid load, progressively removed amyloid from the liver, spleen, and kidneys in acquired and hereditary systemic amyloidosis. Amyloid clearance from the liver was faster and more extensive than from other organs, as previously observed with spontaneous hepatic AA and AL amyloid regression in effectively treated patients (10)(11)(12)(13). This may reflect the fact that the sinusoidal, fenestrated hepatic capillary endothelium allows unhindered access of antibodies and complement proteins from the blood to the extracellular space where the amyloid deposits are located.…”

Marked and Rapid Regression of Hepatic Amyloid Deposition in a Patient with Systemic Light Chain (AL) Amyloidosis after High-dose Melphalan Therapy with Stem Cell Transplantation

“…In patients with familial amyloid polyneuropathy and AA amyloidosis, clear regression of tissue amyloid deposition after treatment is seen in the abdominal fat pad (23) or gastroduodenal mucosa (24,25), and similar findings have been reported in AL amyloidosis patients (8,9). With regard to hepatic amyloidosis, however, regression has only been demonstrated radiographically (26,27), not histopathologically, except for one case in which histological regression was observed 5.5 years after treatment (12). In previous reports, it also took two to four years to confirm the apparent histological regression of AL amyloid in other organs (8,9).…”

Amyloidosis presenting as acute liver failure: a case report

“…However, even without the complication of liver failure, patients with hepatic amyloid deposition secondary to systemic AL carry a poor prognosis. One study, consisting of 130 patients, demonstrated that 5-year survival decreased from 72% to 43% when liver involvement of amyloid was noted [16]. Elevated bilirubin and cardiac involvement are also predictors of poor prognosis, both of which are pertinent to our case as well [17].…”