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See Latest ArticlesDiagnosis, pathogenesis, treatment, and prognosis of hereditary fibrinogen Aα chain amyloidosis
Journal of the American Society of Nephrology, 12/15/08
Gillmore JD et al. – Fibrinogen amyloidosis is predominantly a renal disease characterized by variable penetrance, distinctive histological appearance, proteinuria, and progressive renal impairment. Survival is markedly better than observed with systemic AL amyloidosis, and outcomes with renal replacement therapy are comparable to those for age-matched individuals with nondiabetic renal disease.
Methods- Aim was to describe 71 pts with fibrinogen amyloidosis, prospectively studied at the UK National Amyloidosis Centre
- Median age: 58 yrs; renal involvement led to diagnosis in all cases
- At a median f/u of 4 yrs, extra-renal disease was rare; renal histology was characteristic
- 4 amyloidogenic mutations in fibrinogen (P552H, E540V, T538K, and T525fs) were discovered
- Family history of renal disease was frequently absent
- Median time from presentation to ESRD was 4.6 yrs, and the estimated median pt survival from presentation was 15.2 yrs
- Among 44 pts who reached ESRD, median survival was 9.3 yrs
- 12 renal transplants survived for a median of 6.0 yrs
- 7 grafts failed after median f/u from transplantation of 5.8 yrs
- At censor, the longest surviving graft was 12.2 yr
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