Fact-sheet: Renal amyloidosis

Updated on 10/03/2018 at 9:36 AM

Note : 0/10

Definition

Amyloidosis is a rare disease that can affect a large number of organs in a highly variable manner

Involvement is most often systemic, but localized forms are possible (10

-20%).

More rarely, it can present as an isolated soft-tissue mass mimicking a tumoral lesion.

The etiology remains unknown.

An increase in prevalence is observed in relation to increased life expectancy.

50% of patients with secondary amyloidosis die of end-stage renal failure.

Nephrotic syndrome in 30% of cases without hematuria.

The radiological manifestations of amyloidosis are HIGHLY

VARIED and NONSPECIFIC.

Nevertheless, amorphous and irregular calcifications are sometimes associated with amyloid deposits. The pathophysiology of this calcification process remains poorly understood.
In a suggestive clinical context, these calcifications should prompt inclusion of amyloidosis in the differential diagnosis.
In its localized form, amyloidosis can mimic a tumoral process.

Increased size at disease onset.

Followed by atrophic Kidneys with cortical thinning.

Mass effect.

AL amyloidosis:

AA amyloidosis:

Transthyretin amyloidosis:

liver transplantation, allows suppression of mutated transthyretin synthesis in favor of non-mutated transthyretin.

It is based on the nature of the protein precursor.

To date, at least 24 different proteins have been recognized as causative agents of amyloidosis.

The 4 most common are the amyloid proteins AL, AA, ATTR, and Aβ2M.

AL (amyloid light-chain protein), so-called "primary" amyloidosis, associated with myeloma, Waldenström macroglobulinemia, CLL, lymphoma...

AA: AA amyloidosis (amyloid A protein), so-called secondary or reactive amyloidosis due to inflammation

ATTR: Transthyretin amyloidosis, mutated (hereditary) or senile

ß-2-microglobulin amyloidosis, often associated with chronic renal failure on dialysis.