Adjustment of Eculizumab Dosage Pattern in Patients with Atypical Hemolytic Uremic Syndrome with Suboptimal Response to Standard Treatment Pattern

Monteavaro, C. Garcia; Roselló, Carmen Peralta; Quiroga, Borja; Martín, José María Baltar; Eraso, Lorena Castillo; Moreno, Fernando de Álvaro; Vea, Alberto Martínez; Manzanos, María Teresa Visus-Fernández de

doi:10.1155/2016/7471082

Cited by 2 publications

(1 citation statement)

References 39 publications

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“…Unmonitored dose reduction was examined in 2 patients, with both relapsing [18]. There are also reports of potential breakthrough of the traditional dosing schedule, but which responded to dose increase [24]. Finally, a low cost and low risk method utilized in some studies is home urine dipstick testing for evidence of hemoglobinuria; this method merits further exploration in those at high risk for relapse, and could be measured by patients between scheduled inoffice follow up with physicians.…”

Section: Atypical Husmentioning

confidence: 99%

When to Stop Eculizumab in Complement-Mediated Thrombotic Microangiopathies

Olson

Sulpizio

et al. 2018

Am J Nephrol

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The terminal complement-inhibitor eculizumab has dramatically changed the management of patients with atypical hemolytic uremic syndrome (aHUS), and has also shown promise for treating certain forms of secondary HUS (sHUS), including that caused by drugs and solid-organ/hematopoietic stem cell transplant. While effective, eculizumab is costly and inconvenient. In this review, we evaluate the literature on eculizumab cessation in these diseases to better inform clinicians who consider stopping therapy. Reported relapse rates in aHUS after stopping eculizumab are as high as 30%, suggesting indefinite therapy is reasonable and that patients who choose to stop should be closely monitored. In sHUS, relapse is rare, justifying short courses of eculizumab.

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Section: Atypical Husmentioning

confidence: 99%

When to Stop Eculizumab in Complement-Mediated Thrombotic Microangiopathies

Olson

Sulpizio

et al. 2018

Am J Nephrol

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Atypical hemolytic-uremic syndrome: evolution of treatment and impact of clinical and genetic characteristics on possibility of eculizumab withdrawal

Prokopenko

2022

Nefrologiâ (St.-Peterbg.)

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Atypical hemolytic uremic syndrome (aHUS) is a rare variant of thrombotic microangiopathy (TMA) associated with uncontrolled activation of alternative complement pathway due to mutations in complement regulatory protein genes or antibodies formation to regulators. Clinical manifestations of aHUS can be triggered by infections, sepsis, pregnancy, autoimmune diseases, organ transplantation, and other complement-activating conditions. Previously, the only treatment option for aHUS was plasma therapy – fresh frozen plasma infusions or plasma exchange, but its effectiveness was insufficient. Currently, targeted treatment available – recombinant monoclonal antibodies against complement C5 protein – eculizumab with high efficiency in achieving aHUS remission, renal function recovery, and preventing TMA at kidney transplantation. For a long time, the question of the optimal duration of treatment and the possibility of eculizumab discontinuing remained unresolved. It was shown that aHUS relapses developed in 20-35 % of patients after discontinuation of complement-blocking therapy. The article presents an overview of a large number of studies of eculizumab treatment outcomes and the possibility of its withdrawal, including a French prospective multicenter study that identified risk factors for aHUS relapse after eculizumab discontinuation: the presence of rare variants of complement genes, female gender, increased soluble C5b-9 plasma level. In patients who did not have rare genetic variants, the risk of relapse was less than 5 %. In general, eculizumab discontinuation after achieving complete remission of aHUS and renal function recovery in patients with low risk of recurrence can provide better tolerability of maintenance treatment, and decrease the incidence of infectious complications and the financial burden on the healthcare system.

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Adjustment of Eculizumab Dosage Pattern in Patients with Atypical Hemolytic Uremic Syndrome with Suboptimal Response to Standard Treatment Pattern

Cited by 2 publications

References 39 publications

When to Stop Eculizumab in Complement-Mediated Thrombotic Microangiopathies

When to Stop Eculizumab in Complement-Mediated Thrombotic Microangiopathies

Atypical hemolytic-uremic syndrome: evolution of treatment and impact of clinical and genetic characteristics on possibility of eculizumab withdrawal

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