title: Plasmatherapy in atypical hemolytic uremic syndrome.
abstract: Plasmatherapy has become empirically first-line treatment in atypical hemolytic uremic syndrome (aHUS), although no prospective controlled trials have been conducted. Patients with mutations that induce complete or partial factor H (FH) quantitative deficiency may be controlled by plasma infusions (PI), but plasma exchanges appear more efficient than PI in patients with mutations that result in a mutant dysfunctional FH in the circulation. Early treatment is crucial. Long-term prophylactic plasmatherapy appears more efficient to prevent end-stage renal disease (ESRD) than plasmatherapy only during relapses. However, the longest follow-up with preserved renal function under plasmatherapy is only 6.5 years. Plasmatherapy does not appear to influence the outcome of aHUS with membrane cofactor protein mutation, and its efficacy in patients with factor I, C3, or factor B mutations is suggested by a few reports. We hope complement blockers will offer patients a better chance to avoid ESRD and provide a better quality of life.
authors.affiliation: Assistance Publique-Hôpitaux de Paris, Pediatric Nephrology Department, Université Paris-Diderot, Hôpital Robert Debré, Paris, France. chantal.loirat@rdb.aphp.fr
authors.forename: Chantal
authors.initials: C
authors.lastname: Loirat
authors.forename: Arnaud
authors.initials: A
authors.lastname: Garnier
authors.forename: Anne-Laure
authors.initials: AL
authors.lastname: Sellier-Leclerc
authors.forename: Theresa
authors.initials: T
authors.lastname: Kwon
mesh_terms: D017242:Complement Factor H / Q000235:genetics / Q000276:immunology; D005340:Fibrinogen / Q000235:genetics / Q000276:immunology; D006463:Hemolytic-Uremic Syndrome / Q000235:genetics / Q000276:immunology / Q000628:therapy*; D006801:Humans; D007676:Kidney Failure, Chronic / Q000276:immunology / Q000517:prevention & control; D051916:Membrane Cofactor Protein / Q000235:genetics / Q000276:immunology; D009154:Mutation; D010951:Plasma Exchange; D013997:Time Factors; D016896:Treatment Outcome
chemical_list: D051916:Membrane Cofactor Protein; D017242:Complement Factor H; D005340:Fibrinogen