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Amyloid A (AA) amyloidosis is a rare but life-threatening complication of cystic fibrosis (CF) driven by chronic inflammation. Despite advances in CF care, the clinical features and outcomes of CF-associated AA amyloidosis remain poorly defined. We aimed to characterize this complication through a case series and literature review. PubMed, Scopus, and Web of Science were searched through November 2025 for published cases of biopsy-proven AA amyloidosis in patients with CF. Reported cases were reviewed, and available clinical data were summarized; three additional cases from our center were included. A total of 70 patients were analyzed, including three cases from our center. The median age at CF diagnosis was 45 months, whereas the median age at AA amyloidosis was 21 years. Renal involvement occurred in 69 patients (98.6%), and 74% had nephrotic-range proteinuria. Among 24 patients with treatment data, 11 received RAAS blockade, nine colchicine, five corticosteroids, and three IL-1 or IL-6-targeting biologic therapies. Of the 55 patients with outcome data, 50 (91%) died, with a median survival of 13 months after diagnosis.

CF-associated AA amyloidosis is a devastating complication characterized by predominant renal involvement and poor survival. Regular urinary protein assessment may facilitate earlier diagnosis, while further studies are needed to establish effective disease-modifying therapies.

• AA amyloidosis is a rare complication of cystic fibrosis (CF) driven by chronic inflammation, with renal involvement as the predominant manifestation. • Reported outcomes have historically been poor, but the evidence has been limited to isolated case reports.

• In this largest review to date (70 patients), mortality was 91% with a median survival of 13 months after diagnosis. • We report the first cases of CF-associated AA amyloidosis treated with biologic therapies (anti-IL-1, anti-IL-6), with no meaningful clinical response.