Involvement In Amyloidosis Research Articles

Amyloid cardiomyopathy in systemic non-hereditary amyloidosis. Clinical, echocardiographic and electrocardiographic findings in 30 patients with AA and 24 patients with AL amyloidosis

To underline the role of echocardiography in the detection of cardiac involvement in patients with amyloidosis, physical examination, echocardiography and electrocardiography were performed in 30 patients with AA amyloidosis (amyloid protein A, associated with chronic inflammatory disease, usually without cardiomyopathy) and 24 patients with AL amyloidosis (the immunoglobulin light chain derived type, often associated with cardiomyopathy). All patients had histological confirmation of amyloidosis by rectal or subcutaneous abdominal fat biopsy. The combination of increased thickness of the left ventricular posterior wall and interventricular septum with a low voltage electrocardiographic pattern is highly specific for cardiac amyloidosis and was found in 3/30 (10%) of the AA patients and in 13/24 (54%) of the AL patients. The echocardiographic abnormalities were strongly related to the degree of clinical heart disease, showing mildly or moderately increased wall thickness in the early asymptomatic phase or severe thickening and hypokinesia of the left ventricular posterior wall and interventricular septum in clinically apparent cardiac dysfunction. Echocardiography appears to be a sensitive test for the detection of cardiac involvement in amyloidosis, in symptomatic as well as asymptomatic patients.

Amyloidosis

The radiographic demonstration of lymph node involvement in amyloidosis is rare. We report a case of calcified, enlarged perioartic and mediastinal lymph nodes detected by CT.

Intrahepatic cholestasis with predominant pericentral deposition in systemic amyloidosis

Liver involvement in amyloidosis is rarely associated with intrahepatic cholestasis. The cases recorded in the literature indicate that there is a tendency toward periportal deposition, leading to centrilobular cholestasis. Our case was most interesting in that the amyloid deposition was most severe in the centrilobular area. Presumably there was still sufficient compression of canaliculi at the periportal level to produce obstruction of the bile flow. This would appear to be a less common but distinct variant of systemic amyloidosis with associated obstructive jaundice.

Cardiac amyloidosis imaging

Amyloidosis is a systemic disorder produced by the deposition of insoluble protein fibrils that fold and deposit in the myocardium. Patients with amyloidosis and cardiac involvement have higher mortality than patients without cardiac involvement. The two most prevalent forms of amyloidosis associated with cardiac involvement are AL amyloidosis, due to the deposition of immunoglobulin light chains, and ATTR amyloidosis, due to the deposition of the transthyretin (TTR) protein in mutated or senile form. This article aims to review the different cardiac imaging modalities (echocardiography, cardiac magnetic resonance imaging, nuclear medicine and tomography) that allow to determine the severity of cardiac involvement in patients with amyloidosis, the type of amyloidosis and its prognosis. Finally, we suggest a diagnostic algorithm to determine cardiac involvement in amyloidosis adapted to locally available diagnostic tools, with a practical and clinical approach.