Work

Treatment with CFTR modulators and subsequent remission of AA amyloidosis

Year: 2026

Type: article

Abstract: 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.

Source: Clinical Kidney Journal

DOI: 10.1093/ckj/sfag266

Authors: Rachel McDougall, Christopher Hill

Institution: Belfast City Hospital

Language: English

Topic: Amyloidosis: Diagnosis, Treatment, Outcomes

Subfield: Molecular Biology

Field: Biochemistry, Genetics and Molecular Biology

Domain: Life Sciences

SDG: Good health and well-being

Open Access status: gold

Estimated APC paid: $3,335