Treatment with CFTR modulators and subsequent remission of AA amyloidosis

Abstract

CFTR modulators have been revolutionary for cystic fibrosis patients but their potential influence on AA amyloidosis has not been investigated. This case report is of a gentleman with cystic fibrosis and nephrotic range proteinuria secondary to AA amyloidosis. At five years following commencement of CFTR modulators, he is in sustained, complete remission with normal urine protein excretion and serum amyloid protein levels within the normal range.