Complete Clinical Remission and Subsequent Relapse of Bronchiectasis-Related (AA) Amyloid Induced Nephrotic Syndrome

Abstract

Systemic amyloidosis normally has a dismal prognosis. However, there are several case reports of protracted survival, usually as a response to measures designed to retard the further deposition of amyloid fibrils. In AA amyloid, most commonly associated with inflammatory rheumatological, bowel, and chest diseases, such interventions have had some success, but the dramatic response of complete resolution of nephrotic syndrome as a result of the regular institution of postural chest drainage and antibiotic therapy, in the clinical context of bronchiectasis, has been previously reported only once. In both of our cases, after protracted remission, such therapy was abandoned by the patients, leading both to recurrence of nephrotic syndrome and also eventually to end-stage renal failure requiring dialysis.