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Published on: September 5, 2016

Complement alternative pathway activation in the course of thrombotic microangiopathy associated with adult-onset

Pierre-Louis Carron1, Jean-Charles Cartier, Anne-Sophie Truche

  • 1Service de Néphrologie Dialyse et Transplantation, Centre Hospitalier Universitaire, Grenoble, France.

Transfusion and Apheresis Science : Official Journal of the World Apheresis Association : Official Journal of the European Society for Haemapheresis
|April 9, 2013
PubMed

Atypical haemolytic uraemic syndrome is a rare disease associated which genetic or acquired factors those cause defective regulation of the alternative complement pathway. We report the case of a 46-year-old woman who presented with thrombotic microangiopathy coinciding with a monocyclic evolution of adult-onset Still's disease. Low C3 with decreased FB concentration, associated with normal C4 was present until the thrombotic microangiopathy's resolution, indicative of an excessive production of alternative C3 convertase. She responded to plasma exchange. This observation reinforces the hypothesis for a common pathway in the pathogenesis for both of the diseases, and suggests alternative complement pathway mediation.

Keywords:
Adult-onset Still’s diseaseAlternative complement pathwayPasma exchangeThrombotic microangiopathy

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