An effective treatment of atypical hemolytic uremic syndrome with plasma exchange and eculizumab: A case report

Özyurt, Neslihan; Ayer, Mesut; Ergen, Abdulkadir; Öztürk, Savaş

doi:10.1016/j.transci.2015.01.006

Cited by 6 publications

(4 citation statements)

References 9 publications

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“…Although some cases may present mutations in complement regulatory genes [ 149–151 ], several studies have shown a transient complement activation in TMA of different etiologies, without identifiable genetic abnormalities [ 152 ]. These findings could support the temporary use of complement blockers in LN-related TMA unresponsive to immunosuppressive therapy [ 153 , 154 ], although controlled prospective studies are needed.…”

Section: Special Situationsmentioning

confidence: 75%

Diagnosis and treatment of lupus nephritis: a summary of the Consensus Document of the Spanish Group for the Study of Glomerular Diseases (GLOSEN)

Rojas-Rivera

García-Carro

Ávila

et al. 2023

Clinical Kidney Journal

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Lupus nephritis (LN) is the most frequent serious manifestation of patients with systemic lupus erythematosus (SLE). Up to 60% of SLE patients develop LN, which has a significant impact on their quality of life and prognosis. Recent advances have improved the diagnostic approach to LN, and new drugs that block specific pathways and kidney damage progression have been developed. Several randomized and well-powered clinical trials have confirmed the efficacy of these agents in terms of proteinuria remission and preservation of kidney function in the medium and long term, with an acceptable safety profile and good tolerance. The combination of different therapies allows to reduce the dose and duration of corticosteroids and other potentially toxic therapies and to increase the number of patients achieving complete remission of the disease. This consensus document carried out by the Spanish Group for the Study of Glomerular Diseases (GLOSEN) provides practical and updated recommendations, based on the best available evidence and clinical expertise of participating nephrologists.

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Section: Special Situationsmentioning

confidence: 75%

Diagnosis and treatment of lupus nephritis: a summary of the Consensus Document of the Spanish Group for the Study of Glomerular Diseases (GLOSEN)

Rojas-Rivera

García-Carro

Ávila

et al. 2023

Clinical Kidney Journal

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“…In the absence of eculizumab, the efficacy of plasma exchange and plasma infusion varies, and the duration of therapy is dependent on the treatment response. [900][901][902][903] Data from 31 adult patients (26 treated with plasma therapy and 5 plasma-resistant patients treated with eculizumab) showed recovery of kidney function in approximately 40% of patients given plasma therapy. 895 Adverse pregnancy outcomes, such as preeclampsia, preterm birth, and fetal loss, are higher in patients with active LN.…”

Section: Treatment Of Ln Relapsementioning

confidence: 99%

KDIGO 2021 Clinical Practice Guideline for the Management of GLOMERULAR Diseases

Bobkova¹,

Bulanov²,

Zakharova³

et al. 2022

N&D

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CURRENT CHRONIC KIDNEY DISEASE (CKD) NOMENCLATURE USED BY KDIGOCKD is defined as abnormalities of kidney structure or function, present for >3 months, with implications for health. CKD is classified based on Cause, GFR category (G1-G5), and Albuminuria category (A1-A3), abbreviated as CGA.Green, low risk (if no other markers of kidney disease, no CKD); yellow, moderately increased risk; orange, high risk; red, very high risk. GFR, glomerular filtration rate

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“…Atypical HUS (aHUS) does not include diarrhea and is associated with defective complement regulation, resistance to treatment, and poor prognosis [ 2 ]. Eculizumab, an anti-C5 antibody, is effective in limiting complement activation in patients with aHUS and has recently become a therapeutic option for aHUS [ 3 ]. Investigators have published case reports describing the effectiveness of eculizumab and plasma exchange in the treatment of patients with aHUS [ 3 , 4 ].…”

Section: Introductionmentioning

confidence: 99%

“…Eculizumab, an anti-C5 antibody, is effective in limiting complement activation in patients with aHUS and has recently become a therapeutic option for aHUS [ 3 ]. Investigators have published case reports describing the effectiveness of eculizumab and plasma exchange in the treatment of patients with aHUS [ 3 , 4 ]. Crescentic IgA nephropathy has a poor prognosis, and the initial serum creatinine level may predict renal failure [ 5 ].…”

Section: Introductionmentioning

confidence: 99%

Coexistence of atypical hemolytic uremic syndrome and crescentic IgA nephropathy treated with eculizumab: a case report

Matsumura

Tanaka

Nakamura

et al. 2016

CNCS

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Rapid progression to end-stage renal disease has been reported in a minority of patients with immunoglobulin A (IgA) nephropathy. In particular, crescentic IgA nephropathy has a poor prognosis in patients with a higher initial serum creatinine level. The complement system plays an important role in the pathogenesis of crescentic IgA nephropathy. Atypical hemolytic uremic syndrome (aHUS), which is characterized by thrombotic microangiopathy, is distinct from Shigatoxin-induced HUS and thrombotic thrombocytopenic purpura. aHUS is associated with dysregulation of the alternative complement system. Eculizumab, an anti-C5 antibody, is effective in limiting complement activation in patients with paroxysmal nocturnal hemoglobinuria, aHUS, or refractory IgA nephropathy in some case reports. We herein report the case of a 42-year-old man with acute kidney injury (AKI) clinically and histologically diagnosed with the coexistence of aHUS and crescentic IgA nephropathy. The patient was treated with steroids, plasmapheresis, and hemodialysis; however, eculizumab treatment was initiated on hospital day 21 due to resistance to and dependence on the conventional aggressive therapy. Clinical remission of aHUS was achieved on day 70, but the renal function failed to recover from dialysis dependence. To the best of our knowledge, this is the first report showing the clinical course of a refractory patient with the coexistence of aHUS and crescentic IgA nephropathy treated with eculizumab. This case highlights the clinical importance of early diagnosis and appropriate initiation of eculizumab for the treatment of this type of AKI.

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An effective treatment of atypical hemolytic uremic syndrome with plasma exchange and eculizumab: A case report

Cited by 6 publications

References 9 publications

Diagnosis and treatment of lupus nephritis: a summary of the Consensus Document of the Spanish Group for the Study of Glomerular Diseases (GLOSEN)

Diagnosis and treatment of lupus nephritis: a summary of the Consensus Document of the Spanish Group for the Study of Glomerular Diseases (GLOSEN)

KDIGO 2021 Clinical Practice Guideline for the Management of GLOMERULAR Diseases

Coexistence of atypical hemolytic uremic syndrome and crescentic IgA nephropathy treated with eculizumab: a case report

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