Autoinflammatory & rare systemic diseases

Renal outcomes and survival in amyloidosis associated with familial Mediterranean fever: A longitudinal study

Semin Arthritis RheumPublished Retrospective cohort study

Authors' conclusion

FMF-amyloidosis generally has poor long-term prognosis, significantly influenced by the amyloid burden at diagnosis.

Study context

Population
Patients with Familial Mediterranean fever (FMF)-associated amyloidosis treated between 2000 and 2022
Participants
61 patients
Exposure
Estimated glomerular filtration rate (eGFR) <67 vs ≥67 mL/min/1.73 m² at amyloidosis diagnosis; IL-1 blockers
Outcome
Rate of end-stage renal disease or death during the study period
Methodological detailslongitudinal, Kaplan-Meier analysis, maximally selected rank statistics
  • Longitudinal
  • Kaplan-Meier analysis
  • Maximally selected rank statistics

Main finding

As reported in the abstract

End-stage renal disease or death · mean of 10.8 ± 9.6 years from diagnosis of amyloid nephropathy

Attained the primary outcome

82%

Observational data: associations, not effects.

Further findings

10-year kidney or overall survival by eGFR cutoff at amyloidosis diagnosis

10-year kidney or overall survival by eGFR cutoff at amyloidosis diagnosis

eGFR cutoff <67 mL/min/1.73 m² significantly associated with shorter time to endpoint

Outcome determinants at amyloidosis diagnosis

Lower eGFR and increased concomitant medication usage were significant outcome determinants

IL-1 blocker treatment (13 patients)

No impact on outcomes (Kaplan-Meier)
13

Original publication

Renal outcomes and survival in amyloidosis associated with familial Mediterranean fever: A longitudinal study. Semin Arthritis Rheum. Published 30 January 2025. doi:10.1016/j.semarthrit.2025.152642

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