Fabry's disease natural history, complications and prognosis

Natural History [1]

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Homozygotes

• The symptoms of classic Fabry's disease usually develop in childhood or adolescents , and start with symptoms such as neuropathic pains, angiokeratomas, dyshidrosis, GI symptoms, and etc.
• Without treatment, lifespan of homozygote men will dramatically reduced to fifth decade and the main causes of deaths are renal failure, heart disease or stroke.

Heterozygote

• The symptoms of late-onset Fabry's disease in females and atypical variants are accrue in higher ages and are less severe.
• With out treatment, life span of heterozygote females will be at seventh decade and the main causes of death are cardiac dysfunction, cancer and stroke.

Complications

• Common complications that can develop as a result of Fabry's disease are:
  • Heart problems including cardiomyopathy, Arrhythmia, Heart failure
  • Renal failure (ESRD)
  • Peripheral neuropathy
  • Strokes (such as TIA)
• Complications that can develop as a result of the treatment of Fabry's disease with enzyme replacement therapy are:
  • Infusion reactions: rigors, fever, etc.
  • Seroconversion: presents of anti-bodies may lower the efficacy of ERT.

Prognosis

• The prognosis of Fabry's disease improves with treatment. Without treatment, Fabry's disease will result in reduced life expectancy.
  

References

[1] 1-903539-03-XFabry, Hermann, et al. Fabry disease : perspectives from 5 years of FOS. Oxford: PharmaGenesis, 2006. Print.