Complement alternative pathway activation in the course of thrombotic microangiopathy associated with adult-onset Still's disease.
Transfus Apher Sci
; 49(3): 533-4, 2013 Dec.
Article
en En
| MEDLINE
| ID: mdl-23562215
ABSTRACT
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.
Palabras clave
Texto completo:
1
Bases de datos:
MEDLINE
Asunto principal:
Intercambio Plasmático
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Enfermedad de Still del Adulto
/
Vía Alternativa del Complemento
Tipo de estudio:
Prognostic_studies
/
Risk_factors_studies
Límite:
Female
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Humans
/
Middle aged
Idioma:
En
Revista:
Transfus Apher Sci
Asunto de la revista:
HEMATOLOGIA
Año:
2013
Tipo del documento:
Article