COMPLETE CLINICAL REMISSION AND SUBSEQUENT RELAPSE OF BRONCHIECTASIS-RELATED (AA) AMYLOID INDUCED NEPHROTIC SYNDROME

Systemic amyloidosis normally has a dismal prognosis. However, there a re several case reports of protracted survival, usually as a response to measures designed to retard the further deposition of amyloid fibri ls. In AA amyloid, most commonly associated with inflammatory rheumato logical, bowel, and chest diseases, such interventions have had some s uccess, but the dramatic response of complete resolution of nephrotic syndrome as a result of the regular institution of postural chest drai nage 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, lead ing both to recurrence of nephrotic syndrome and also eventually to en d-stage renal failure requiring dialysis.