Primary amyloidosis (AL) as a cause of nephrotic syndrome.
AL amyloidosis is a rare systemic disease resulting from tissue accumulation of amyloid fibrils derived from monoclonal immunoglobulin light chains. It can disrupt the tissue architecture and consequently cause organ dysfunction. The prognosis is poor with a median survival of 13 months in untreated patients. By illustrating the case of a patient whose AL amyloidosis was detected after presenting a nephrotic syndrome, the characteristics of the disease are reviewed as well as diagnostic criteria and current available therapeutics.
Bataille
Y
Y
Department of nephrology and hypertension, University Hospital Liège, Domaine universitaire du Sart Tilman- B.35, B 4000 Liege 1, Belgique.
Bovy
C
C
Lancellotti
P
P
Melchior
V
V
Delbecque
K
K
Beguin
Y
Y
Krzesinski
J M
JM
D000686:Amyloidosis / Q000150:complications* / Q000175:diagnosis*; D001707:Biopsy, Needle; D018450:Disease Progression; D017809:Fatal Outcome; D006801:Humans; D007150:Immunohistochemistry; D007677:Kidney Function Tests; D008297:Male; D008875:Middle Aged; D009102:Multiple Organ Failure; D009404:Nephrotic Syndrome / Q000175:diagnosis / Q000209:etiology* / Q000628:therapy; D012720:Severity of Illness Index; D018608:Ultrasonography, Doppler