Introduction Hereditary fibrinogen A alpha-chain (AFib) amyloidosis is characterised by progressive proteinuric chronic kidney disease (CKD) and early progression to end-stage renal disease (ESRD). A large series published in 2009 showed renal survival <5 years from diagnosis and renal transplant survival of <10 years. Since then, advances in diagnostic techniques and nephroprotective care have transformed both nephrology and amyloidosis. We evaluated contemporary diagnostic and renal outcomes in the largest cohort of patients with AFib amyloidosis to date. Methods 194 patients with confirmed AFib amyloidosis followed at a large national centre diagnosed between 1993 and 2025 were included. Patients were stratified by era of diagnosis, pre- or post-2009. Diagnostic approaches, renal outcomes, transplant outcomes, and patient survival were evaluated. Results Median age at diagnosis was 60 years with mean eGFR of 37 ml/min/1.73m2. Proteomics-based amyloid typing became the predominant diagnostic modality post-2009 (60.5%). 62.8% progressed to ESRD. The mean annual rate of eGFR decline was 6.7 ml/min/1.73m2 pre-2009 and 4.8 ml/min/1.73m2 post-2009. Renal survival improved post-2009, particularly amongst patients with CKD stage IIIA/B at diagnosis. Median renal allograft survival improved from 7.7 years pre-2009 to 15.5 years post-2009 (p=0.006; HR [95% CI] 4.5 [1.2 to 22.6]). Overall patient survival did not differ significantly between eras (median 12 years; p=0.69, log-rank). Conclusions Renal and allograft survival have improved since 2009, likely reflecting advances in supportive nephrology care. Isolated kidney transplantation is associated with favourable long-term outcomes. Despite improvements in renal survival, overall survival remains unchanged, underscoring the need for effective amyloid-targeted therapies.
Introduction Kidney cyst infections are serious complications in autosomal dominant polycystic kidney disease (ADPKD). Kidney transcatheter arterial embolization (TAE) reduces kidney volume in this population. We tested the hypothesis that TAE could prevent kidney cyst infections. Methods This retrospective cohort study included 416 ADPKD patients who underwent kidney TAE, including 70 with a prior kidney cyst infection. The primary outcome was the annual frequency of kidney cyst infections. Secondary outcomes included hospitalization duration. Risk factors for post-TAE infection recurrence were identified by multivariable linear regression. Results In the primary cohort, the frequency of cyst infections decreased from 0.291 episodes/year pre-TAE to 0.087 post-TAE (p<0.001). In the subgroup with a prior infection, the frequency decreased from 1.73 ± 1.18 to 0.49 ± 0.96 episodes/year (p<0.001), and hospitalization days decreased from 34.1 ± 26.1 to 9.90 ± 24.4 days/year (p<0.001). The reduction effect was pronounced in patients with a greater total kidney volume reduction rate (TKV-RR); no infections were observed in patients who achieved a TKV-RR >75%. Multivariable analysis revealed pre-TAE infection frequency (β=0.23; p=0.017), a lower TKV-RR (β=-0.01; p=0.034), and antibiotic use at TAE (β=0.68; p=0.007) as independent risk factors for post-TAE infection recurrence. Even among patients who consistently used antibiotics, the infection frequency decreased post-TAE. Conclusions Kidney TAE is a promising technique for reducing kidney cyst infections in ADPKD patients. TAE should be performed after full remission without antibiotic therapy, but TAE reduces cyst infection even in consistent antibiotic users. Early, extensive embolization might be particularly beneficial in high-risk patients.
