Early initiation of eculizumab therapy for <i>Streptococcus pneumoniae</i>–associated hemolytic uremic syndrome

See, Jhoclay; Matar, Raed Bou; Baloğlu, Orkun; Latifi, Samir; Talati, Ravi; Agarwal, Hemant

doi:10.1002/pbc.28589

Cited by 3 publications

(5 citation statements)

References 17 publications

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“…See et al used eculizumab in a 2-year-old girl on the third day after P-HUS diagnosis. The PLT level started to rise after 3 days (day 6 after P-HUS), and she required KRT for 15 days in total [ 9 ]. Jeantet et al used eculizumab in a 55-year-old man after unsuccessful PE.…”

Section: Discussionmentioning

confidence: 99%

Eculizumab use in patients with pneumococcal-associated hemolytic uremic syndrome and kidney outcomes

Konopásek

Zieg

2023

Pediatr Nephrol

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Background Streptococcus pneumoniae-associated hemolytic uremic syndrome (P-HUS) is a rare and severe disease. Only a few reports have been published about eculizumab use in P-HUS. Methods We analyzed demographic, clinical, and laboratory data of patients with P-HUS from our center. Results The cohort consisted of 4 females and 3 males. All patients had pneumonia. Four were given eculizumab (days 1–3). The eculizumab group required a shorter duration of dialysis and mechanical ventilation (medians 20 vs. 28.5 and 30 vs 38.5 days, respectively) compared with the non-eculizumab group, but this was still much longer than normally reported; the thrombocytopenia resolution was similar in both groups (medians 10 vs. 8 days). Chronic kidney disease (CKD) was correlated with the duration of dialysis and mechanical ventilation duration at 1 year (r = 0.797, P = 0.032 and r = 0.765, P = 0.045) and last follow-up (r = 0.807, P = 0.028 and r = 0.814, P = 0.026, respectively); our scoring system showed even stronger correlations (r = 0.872, P = 0.011 and r = 0.901, P = 0.0057, respectively). The eculizumab group showed slightly better 1-year and last follow-up CKD stage (2.75 vs. 3, P = 0.879 and 2.5 vs. 3.67, P = 0.517). Conclusions Despite the fact that the eculizumab group showed better outcomes, eculizumab does not seem to improve the course of P-HUS compared with previous reports. Kidney outcomes are strongly correlated with the duration of dialysis and mechanical ventilation duration. Graphical Abstract

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Section: Discussionmentioning

confidence: 99%

Eculizumab use in patients with pneumococcal-associated hemolytic uremic syndrome and kidney outcomes

Konopásek

Zieg

2023

Pediatr Nephrol

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“…They came off renal replacement therapy within a month of initiation of eculizumab and had normal to near normal renal function on long term follow-up [66]. Similarly, eculizumab has demonstrated good response in isolated case reports of both pediatric and adult Sp-HUS patients [96,114,115]. Two pediatric Sp-HUS patients, with low C3 level in one patient and low C3 and C4 levels in another patient, with no detectable abnormalities in the complement pathway received eculizumab therapy in the acute phase.…”

Section: Eculizumabmentioning

confidence: 99%

“…Sp-HUS patients demonstrate unregulated complement activity, and there may be a role for the use of eculizumab in these patients, as it blocks the activation of the terminal complement pathway and prevents complement mediated damage. Case series and isolated case reports have reported good outcomes in severe Sp-HUS cases following eculizumab administration [66,96,114,115]. Eculizumab was administered to 3 patients in a case series of seven Sp-HUS patients between 1996 and 2019 [66].…”

Section: Eculizumabmentioning

confidence: 99%

“…Both patients received 4 doses of eculizumab. They demonstrated good renal recovery, renal replacement therapy was stopped within 15-30 days, and they had near normal to normal renal function on long term follow up [96,115]. In another case report of a 53 year old adult Sp-HUS patient, with normal complement factor levels and no defects of complement-regulating genes; eculizumab was initiated after failure of conventional therapy and plasma exchange [114].…”

Section: Eculizumabmentioning

confidence: 99%

See 1 more Smart Citation

Streptococcus Pneumoniae-Associated Hemolytic Uremic Syndrome in the Era of Pneumococcal Vaccine

Agarwal

Latifi

2021

Pathogens

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Streptococcus pneumoniae-associated hemolytic uremic syndrome (Sp-HUS) is a serious complication of invasive pneumococcal disease that is associated with increased mortality in the acute phase and morbidity in the long term. Recently, Sp-HUS definition has undergone revision and cases are categorized as definite, probable, and possible, based on less invasive serological investigations that evaluate Thomsen-Friedenreich crypt antigen (T-antigen) activation. In comparison to the pre-vaccine era, Sp-HUS incidence seems to be decreasing after the introduction of 7-serotype valence and 13-serotype valence pneumococcal vaccines in 2000 and 2010, respectively. However, Sp-HUS cases continue to occur secondary to vaccine failure and emergence of non-vaccine/replacement serotypes. No single hypothesis elucidates the molecular basis for Sp-HUS occurrence, although pneumococcal neuraminidase production and formation of T-antigen antibody complexes on susceptible endothelial and red blood cells continues to remain the most acceptable explanation. Management of Sp-HUS patients remains supportive in nature and better outcomes are being reported secondary to earlier recognition, better diagnostic tools and improved medical care. Recently, the addition of eculizumab therapy in the management of Sp-HUS for control of dysregulated complement activity has demonstrated good outcomes, although randomized clinical trials are awaited. A sustained pneumococcal vaccination program and vigilance for replacement serotypes will be the key for persistent reduction in Sp-HUS cases worldwide.

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“…However, plasma exchange is controversial since IgM antibody against the Thomsen–Friedenreich antigen may result in possible increased polyagglutination and aggravation of the microangiopathic phenomenon [ 62 ]. There are case reports of the use of complement inhibitor eculizumab in Sp-HUS with good response [ 67 ].…”

Section: Streptococcus Pneumoniae -Associated Hemolytic Uremic Syndromementioning

confidence: 99%

Thrombotic microangiopathy in children

et al. 2022

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The syndrome of thrombotic microangiopathy (TMA) is a clinical-pathological entity characterized by microangiopathic hemolytic anemia, thrombocytopenia, and end organ involvement. It comprises a spectrum of underlying etiologies that may differ in children and adults. In children, apart from ruling out shigatoxin-associated hemolytic uremic syndrome (HUS) and other infection-associated TMA like Streptococcus pneumoniae -HUS, rare inherited causes including complement-associated HUS, cobalamin defects, and mutations in diacylglycerol kinase epsilon gene must be investigated. TMA should also be considered in the setting of solid organ or hematopoietic stem cell transplantation. In this review, acquired and inherited causes of TMA are described with a focus on particularities of the main causes of TMA in children. A pragmatic approach that may help the clinician tailor evaluation and management is provided. The described approach will allow for early initiation of treatment while waiting for the definitive diagnosis of the underlying TMA.

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Early initiation of eculizumab therapy for Streptococcus pneumoniae–associated hemolytic uremic syndrome

Abstract: Streptococcus pneumoniae is the most common infectious cause of nondiarrheal hemolytic uremic syndrome (HUS). 1 S. pneumoniaeassociated hemolytic uremic syndrome (Sp-HUS) patients have higher morbidity and mortality than diarrhea-associated HUS. 1,2 Manage

Cited by 3 publications

References 17 publications

Eculizumab use in patients with pneumococcal-associated hemolytic uremic syndrome and kidney outcomes

Eculizumab use in patients with pneumococcal-associated hemolytic uremic syndrome and kidney outcomes

Streptococcus Pneumoniae-Associated Hemolytic Uremic Syndrome in the Era of Pneumococcal Vaccine

Thrombotic microangiopathy in children

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