What clinical syndromes should suggest the presence of systemic AL amyloidosis?
| Nephrotic syndrome | Autonomic neuropathy (orthostatic hypotension, gastric atony) |
| Congestive heart failure | CTS (especially if claw hand) |
| Peripheral neuropathy | Hepatic disease |
The most common initial clinical manifestation is nephrotic syndrome. The major sign distinguishing it from other causes of nephrosis is the finding of a monoclonal protein in the serum or urine (electrophoresis and immunofixation should be done). Although overt congestive heart failure can occur in up to one-third of patients, amyloid deposits in the heart are eventually seen on imaging in 90%. Peripheral neuropathy clinically resembles the neuropathy seen in diabetes, including the chronic course. Autonomic neuropathy may be superimposed on peripheral neuropathy or occur alone. A history of CTS is a very important clue to the presence of amyloidosis. It is typically bilateral, and surgical release may not provide complete relief.
