ABSTRACT
More than 50% of primary systemic amyloidosis (AL) patients have renal involvement at diagnosis. Patients usually present with nephrotic syndrome or proteinuria. Elevation in serum creatinine is often not prominent at presentation, but end-stage renal failure can occur during the course of the disease and some patients eventually require dialysis. The aim of therapy is to preserve and possibly to restore renal function, but renal impairment itself limits the therapeutic opportunities. This chapter reviews the clinical records and studies the presentation and outcome of 409 consecutive AL patients who had renal involvement at diagnosis and were referred to the Amyloid Center in Pavia between June 1984 and December 2003. Renal involvement was defined as 24 hour urine protein >0.5 g/24 hours, predominantly albumin. Nearly one fifth of AL patients presenting with renal involvement eventually progress to dialysis. A relatively low serum creatinine concentration is associated to a dialysis rate greater than 10 per 100 person-year.
