Successful Treatment of the Postpartum Atypical Hemolytic Uremic Syndrome With Eculizumab

Zschiedrich, Stefan; Prager, Eric Peter; Kuehn, E. Wolfgang

doi:10.7326/0003-4819-159-1-201307020-00023

Cited by 31 publications

(21 citation statements)

References 5 publications

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“…Remissions were achieved in patients with and without identified complement regulatory deficiencies. Subsequent case reports about eculizumab in native kidneys confirmed these observations [63][64][65][66][67][68][69][70][71][72][73][74][75].…”

Section: Eculizumab --First Experiences Case Reports and Retrospectisupporting

confidence: 52%

Pharmacological treatment of atypical hemolytic-uremic syndrome

Vester¹,

Mache

2013

Expert Opinion on Orphan Drugs

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Section: Eculizumab --First Experiences Case Reports and Retrospectisupporting

confidence: 52%

Pharmacological treatment of atypical hemolytic-uremic syndrome

Vester¹,

Mache

2013

Expert Opinion on Orphan Drugs

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“…The use of eculizumab in P-aHUS has only been reported anecdotally and there is no evidence to guide treatment [4][5][6][7][8]. Here, we report our experience of a patient with P-aHUS treated with eculizumab and the successful discontinuation of this therapy.…”

Section: Introductionmentioning

confidence: 72%

“…To date, there are only five reports of P-aHUS treated with eculizumab in the literature [4][5][6][7][8] (Table 1). The dosing and timing of treatment varied, but all patients showed complete hematological response and renal function recovery after eculizumab therapy with no reports of serious adverse events.…”

Section: Case Reportmentioning

confidence: 91%

Eculizumab in pregnancy-associated atypical hemolytic uremic syndrome: insights for optimizing management

et al. 2015

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Pregnancy-associated atypical hemolytic uremic syndrome is a systemic disease associated with high morbidity and mortality rates, caused by dysregulation of the alternative complement pathway, leading to uncontrolled complement activation resulting in thrombotic microangiopathy. This condition can be effectively treated by anti-C5 therapy, which controls complement activation. Treatment can be safely discontinued after complete remission and resolution of the precipitating cause, especially in patients with a low-risk genetic profile.

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“…There are only few reports of pregnancy-associated aHUS treated with eculizumab in the literature and treatment duration varied. Factor H mutation is also associated with high risk of relapse and poor prognosis [13, 14, 15, 16]. We performed complement testing on our patient and identified polymorphic variants which are associated with aHUS and have been described previously.…”

Section: Discussionmentioning

confidence: 95%

“…However, we did kidney biopsy as soon as possible in order to eliminate other diagnoses, and then the rest of the pregnancy continued without any fetal and maternal problems until the 31st week of pregnancy. Pregnancy-associated aHUS is associated with high maternal mortality and severe long-term morbidity and more than half of all patients develop end-stage renal disease in less than 1 month, most cases (79%) developing in the postpartum period [12, 13, 14]. …”

Section: Discussionmentioning

confidence: 99%

Pregnant Woman with Atypical Hemolytic Uremic Syndrome Delivered a Healthy Newborn under Eculizumab Treatment

Demir

Yazıcı

Kiliçaslan

et al. 2016

Case Rep Nephrol Dial

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Pregnancy-associated thrombotic microangiopathy is a very rare condition; however, it significantly increases fetal or maternal morbidity and mortality. Pregnancy may trigger atypical hemolytic uremic syndrome (aHUS) or thrombotic thrombocytopenic purpura. The risk for pregnancy-associated aHUS is highest during the second pregnancy. The outcome is usually poor with 50–60% mortality; renal dysfunction and hypertension are the rule in those who survive the acute episode. After the development of complement regulation mechanisms and aHUS pathogenesis, eculizumab has been widely used as a first-line treatment in aHUS. Eculizumab has been produced to minimize immunogenicity and Fc-mediated functions, including recruitment of inflammatory cells and complement activation, and using eculizumab in first-line treatment improves kidney function. Recent studies showed that early diagnosis and rapid use of eculizumab in first-line treatment improve outcomes. We demonstrate a case with pregnancy-triggered aHUS occurring in the second trimester, who was successfully treated and delivered a healthy baby under eculizumab treatment.

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Successful Treatment of the Postpartum Atypical Hemolytic Uremic Syndrome With Eculizumab

Cited by 31 publications

References 5 publications

Pharmacological treatment of atypical hemolytic-uremic syndrome

Pharmacological treatment of atypical hemolytic-uremic syndrome

Eculizumab in pregnancy-associated atypical hemolytic uremic syndrome: insights for optimizing management

Pregnant Woman with Atypical Hemolytic Uremic Syndrome Delivered a Healthy Newborn under Eculizumab Treatment

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