Long-term remission of atypical HUS with anti-factor H antibodies after cyclophosphamide pulses View Full Text


Ontology type: schema:ScholarlyArticle     


Article Info

DATE

2013-07-19

AUTHORS

Gwenaëlle Sana, Marie-Agnès Dragon-Durey, Marina Charbit, Karim Bouchireb, Caroline Rousset-Rouvière, Etienne Bérard, Rémi Salomon, Véronique Frémeaux-Bacchi, Patrick Niaudet, Olivia Boyer

ABSTRACT

BackgroundAnti-complement factor H (CFH) autoantibody (Ab)-associated atypical hemolytic uremic syndrome (aHUS) has a poor prognosis, but no consensus exists on its treatment.MethodsWe report the follow-up of four children with anti-CFH Ab (8,000 to >32,000 arbitrary units)-associated aHUS after plasma exchanges (PEs), prednisone, and cyclophosphamide pulse therapy with the evolution of anti-CFH Ab titers and kidney function.ResultsPatient 1 received PEs + prednisone + cyclophosphamide pulses after two relapses following PEs and then PEs + rituximab. The other three patients were treated with PEs + prednisone + cyclophosphamide pulses as a first-line therapy. In our four patients, the induction protocol combining PEs + prednisone + cyclophosphamide pulses led to a rapid and sustained remission up to 6 years, 4 years and 4 months without any maintenance therapy. Kidney function was normal and anti-CFH Ab titer decreased, but remained detectable during remission without any clinical or biological signs of relapse.ConclusionsWe demonstrate the long-term efficiency and safety of cyclophosphamide pulses combined with PEs and prednisone in anti-CFH Ab-associated aHUS leading to a prolonged decrease in anti-CFH Ab titers and prevention of relapses without the need for maintenance therapy. More... »

PAGES

75-83

Identifiers

URI

http://scigraph.springernature.com/pub.10.1007/s00467-013-2558-9

DOI

http://dx.doi.org/10.1007/s00467-013-2558-9

DIMENSIONS

https://app.dimensions.ai/details/publication/pub.1040045929

PUBMED

https://www.ncbi.nlm.nih.gov/pubmed/23868108


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29 schema:description BackgroundAnti-complement factor H (CFH) autoantibody (Ab)-associated atypical hemolytic uremic syndrome (aHUS) has a poor prognosis, but no consensus exists on its treatment.MethodsWe report the follow-up of four children with anti-CFH Ab (8,000 to >32,000 arbitrary units)-associated aHUS after plasma exchanges (PEs), prednisone, and cyclophosphamide pulse therapy with the evolution of anti-CFH Ab titers and kidney function.ResultsPatient 1 received PEs + prednisone + cyclophosphamide pulses after two relapses following PEs and then PEs + rituximab. The other three patients were treated with PEs + prednisone + cyclophosphamide pulses as a first-line therapy. In our four patients, the induction protocol combining PEs + prednisone + cyclophosphamide pulses led to a rapid and sustained remission up to 6 years, 4 years and 4 months without any maintenance therapy. Kidney function was normal and anti-CFH Ab titer decreased, but remained detectable during remission without any clinical or biological signs of relapse.ConclusionsWe demonstrate the long-term efficiency and safety of cyclophosphamide pulses combined with PEs and prednisone in anti-CFH Ab-associated aHUS leading to a prolonged decrease in anti-CFH Ab titers and prevention of relapses without the need for maintenance therapy.
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35 schema:keywords Ab
36 Ab titers
37 ConclusionsWe
38 H antibodies
39 HUS
40 MethodsWe
41 ResultsPatient 1
42 anti-factor H antibodies
43 antibodies
44 atypical HUS
45 atypical hemolytic uremic syndrome
46 autoantibodies
47 biological signs
48 children
49 consensus
50 cyclophosphamide pulse therapy
51 cyclophosphamide pulses
52 decrease
53 efficiency
54 evolution
55 exchange
56 factor H autoantibodies
57 first-line therapy
58 function
59 hemolytic uremic syndrome
60 induction
61 kidney function
62 long-term efficiency
63 long-term remission
64 maintenance therapy
65 months
66 need
67 patients
68 plasma exchange
69 poor prognosis
70 prednisone
71 prevention
72 prevention of relapse
73 prognosis
74 pulse therapy
75 pulses
76 relapse
77 remission
78 rituximab
79 safety
80 signs
81 sustained remission
82 syndrome
83 therapy
84 titers
85 treatment
86 uremic syndrome
87 years
88 schema:name Long-term remission of atypical HUS with anti-factor H antibodies after cyclophosphamide pulses
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