What are the treatments for aHUS?
Although plasma exchange and plasma infusion are sometimes used to manage aHUS, these therapies have not been studied in well-controlled trials.1
Historic Management Approaches2
Plasma exchange or plasma infusion is sometimes used by doctors to manage aHUS. During this process a plasma infusion is given over several hours at a specialized treatment center.3,6
However, plasma does not treat the underlying disease and no clinical studies have shown that plasma exchange therapy is safe or effective in treating aHUS.2,3-5 Plasma exchange/infusion is not a cure and symptoms may return over time.2,3,6,7
Dialysis or a kidney transplant may be needed for aHUS patients as a result of the damage caused by the disease. However, dialysis and kidney transplants have significant risks associated with them, and neither will actually treat or cure aHUS.8
As the understanding of the underlying causes of rare diseases such as aHUS has improved, researchers are finding new, more effective and safer treatments. Talk with your doctor about a management plan that works for you and contains frequent follow-up or monitoring of your rare disease.
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1. Laurence J. Atypical hemolytic uremic syndrome (aHUS): making the diagnosis. Clin Adv Hematol Oncol. 2012;10(suppl 17):1-12. 2. Legendre CM, Licht C, Muus P, et al. N Engl J Med. 2013;368:2169-2181. 3. Sellier-Leclerc A-L, Frémeaux-Bacchi V, Dragon-Durey MA, et al; French Society of Pediatric Nephrology. Differential impact of complement mutations on clinical characteristics in atypical hemolytic uremic syndrome. J Am Soc Nephrol. 2007;18:2392-2400. 4. Noris M, Mescia F, Remuzzi G. STEC-HUS, atypical HUS and TTP are all diseases of complement activation. Nat Rev Nephrol. 2012;8:622-633. 5. Noris M, Caprioli J, Bresin E, et al. Relative role of genetic complement abnormalities in sporadic and familial aHUS and their impact on clinical phenotype. Clin J Am Soc Nephrol. 2010;5:1844-1859. 6. Caprioli J, Noris M, Brioschi S, et al; for the International Registry of Recurrent and Familial HUS/TTP. Genetics of HUS: the impact of MCP, CFH, and IF mutations on clinical presentation, response to treatment, and outcome. Blood. 2006;108:1267-1279. 7. Sarode R, Bandarenko N, Brecher ME, et al. Thrombotic thrombocytopenic purpura: 2012 American Society for Apheresis (ASFA) consensus conference on classification, diagnosis, management, and future research. J Clin Apher. 2013; DOI: 10.1002/jca.21302. [Epub ahead of print]. 8. Cofiell R, Kukreja A, Bedard K, et al. Poster presented at the 55th Annual Meeting of the American Society of Hematology; December 7-10, 2013; New Orleans, LA. Abstract 2184.