Treatment with CFTR modulators and subsequent remission of AA amyloidosis.

Cystic fibrosis transmembrane conductance regulator (CFTR) modulators have been revolutionary for cystic fibrosis patients. Their potential influence on AA amyloidosis has not been investigated. In AA amyloidosis, patients develop amyloid deposits of serum A amyloid, in the context of a chronic inflammatory state. This case report is of a gentleman with cystic fibrosis and nephrotic range proteinuria secondary to AA amyloidosis. At 5 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.