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

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

doi:10.1007/s00467-013-2558-9

Cited by 37 publications

(33 citation statements)

References 28 publications

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“…Plasma exchanges are often applied in combination with immune adsorption and/or immunosuppressive therapy (such as corticosteroids, pulse cyclophosphamide treatment or targeted B cell depletion with rituximab). Available clinical data suggest a good efficacy of plasma exchanges and immunosuppression in factor H autoantibody-associated aHUS [63,64,67,[75][76][77]. Similarly, factor H-associated DDD was successfully treated [51].…”

Section: Treatment Optionsmentioning

confidence: 92%

Autoantibodies to complement components in C3 glomerulopathy and atypical hemolytic uremic syndrome

et al. 2014

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The alternative pathway of complement is implicated in the pathogenesis of several renal diseases, such as atypical hemolytic uremic syndrome, dense deposit disease and other forms of C3 glomerulopathy. The underlying complement defects include genetic and/or acquired factors, the latter in the form of autoantibodies. Because the autoimmune forms require a specific treatment, in part different from that of the genetic forms, it is important to detect the autoantibodies as soon as possible and understand their characteristics. In this overview, we summarize the types of anti-complement autoantibodies detected in such diseases, i.e. autoantibodies to factor H, factor I, C3b, factor B and those against the C3 convertases (C3 nephritic factor and C4 nephritic factor). We draw attention to newly described autoantibodies and their characteristics, and highlight similarities and differences in the autoimmune forms of these diseases.

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Section: Treatment Optionsmentioning

confidence: 92%

Autoantibodies to complement components in C3 glomerulopathy and atypical hemolytic uremic syndrome

et al. 2014

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“…Using a threshold of > 2000 AU/ml [50,52] significantly increased the probability of a relapse-free survival, from 46 to 87 % at last follow-up [52]. Four patients have been reported who went into remission after a short cyclophosphamide pulse therapy (0.5 g/1.73 m 2 × 2 in three patients, 1 g/ 1.73 m 2 × 5 in one patient resistant to PE + rituximab) combined with PE and prednisone, and had full renal recovery up to 6 years, 4 years, and 4 months without any maintenance therapy [108]. Although experience is limited, eculizumab appears to be effective in anti-CFH antibody-HUS [93,[109][110][111].…”

Section: Education Information Cardmentioning

confidence: 94%

An international consensus approach to the management of atypical hemolytic uremic syndrome in children

et al. 2015

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Atypical hemolytic uremic syndrome (aHUS) emerged during the last decade as a disease largely of complement dysregulation. This advance facilitated the development of novel, rational treatment options targeting terminal complement activation, e.g., using an anti-C5 antibody (eculizumab). We review treatment and patient management issues related to this therapeutic approach. We present consensus clinical practice recommendations generated by HUS International, an international expert group of clinicians and basic scientists with a focused interest in HUS. We aim to address the following questions of high relevance to daily clinical practice: Which complement investigations should be done and when? What is the importance of anti-factor H antibody detection? Who should be treated with eculizumab? Is plasma exchange therapy still needed? When should eculizumab therapy be initiated? How and when should complement blockade be monitored? Can the approved treatment schedule be modified? What approach should be taken to kidney and/or combined liver-kidney transplantation? How should we limit the risk of meningococcal infection under complement blockade therapy? A pressing question today regards the treatment duration. We discuss the need for prospective studies to establish evidence-based criteria for the continuation or cessation of anticomplement therapy in patients with and without identified complement mutations.

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“…The relapse rates for RTX and cyclophosphamide were 4/13 (31%) and 3/29 (10.5%), respectively. Cyclophosphamide can give good results [17] in patients resistant to induction therapy with PE, steroids, and RTX. Hence, PE and steroids associated with immunosuppressive therapy (cyclophosphamide pulses or rituximab) have been proposed as first-line therapy for induction in patients with anti-CFHAb-related aHUS.…”

Section: Discussionmentioning

confidence: 99%

Efficacy of rituximab and plasmapharesis in an adult patient with antifactor H autoantibody-associated hemolytic uremic syndrome

et al. 2016

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Antifactor H antibody (anti-CFHAb) is found in 6% to 25% cases of atypical hemolytic uremic syndrome (aHUS) in children, but has been only exceptionally reported in adults. There is no consensus about the best treatment for this type of aHUS. We report the case of an adult patient treated successfully with plasma exchange (PE), steroids, and rituximab.A 27-year-old Caucasian male presented to hospital with anemia, thrombocytopenia, and acute renal failure. One week earlier, he had digestive problems with diarrhea. The diagnosis of anti-CFHAb-associated aHUS (82,000 AU/mL) without CFHR gene mutations was established.He received Rituximab 375 mg/m2 (4 pulses) with PE and steroids. This treatment achieved renal and hematological remission at day (D) 31 and negative anti-CFHAb at D45 (<100 AU/mL). At D76, a fifth rituximab pulse was performed while CD19 was higher than 10/mm3. Steroids were stopped at month (M) 9. The patient has not relapsed during long-term follow-up (M39).Rituximab therapy can be considered for anti-CFHAb-associated aHUS. Monitoring of anti-CFHAb titer may help to guide maintenance therapeutic strategies including Rituximab infusion.

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

Abstract: We 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.

Cited by 37 publications

References 28 publications

Autoantibodies to complement components in C3 glomerulopathy and atypical hemolytic uremic syndrome

Autoantibodies to complement components in C3 glomerulopathy and atypical hemolytic uremic syndrome

An international consensus approach to the management of atypical hemolytic uremic syndrome in children

Efficacy of rituximab and plasmapharesis in an adult patient with antifactor H autoantibody-associated hemolytic uremic syndrome

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