SynthesisClinical and experimental medicine2019
Eculizumab in refractory catastrophic antiphospholipid syndrome: a case report and systematic review of the literature.
Synthesis in Clinical and experimental medicine, 2019. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 23 papers, 2 of them syntheses that pooled it.
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Who cites it
23 citing papers in PubMed, 2 syntheses or guidelines pooled it, 56 citations in OpenAlex.
- Immunomodulating Therapies in Acute Myocarditis and Recurrent/Acute Pericarditis.Frontiers in medicine · 2022Pooled it
- Therapeutic Targeting of the Complement System: From Rare Diseases to Pandemics.Pharmacological reviews · 2021Pooled it
- Catastrophic Antiphospholipid Syndrome in a Young Female Complicated by Systemic Lupus Erythematosus and Left Atrial Myxoma: A Rare Case Report.Clinical case reports · 2026Article
- Catastrophic Antiphospholipid Syndrome: A Review of Current Evidence and Future Management Practices.Cureus · 2024Review
- Eculizumab therapy and complement regulation in a case of resistant catastrophic antiphospholipid syndrome.BMJ case reports · 2024Article
- Article
- An update on the biologics for the treatment of antiphospholipid syndrome.Frontiers in immunology · 2023Review
- Cardiac arrest in the setting of probable catastrophic antiphospholipid syndrome in young patient with a history of COVID infection and polyglandular disorder-Case report.SAGE open medical case reports · 2023Article
- Biological therapy in systemic lupus erythematosus, antiphospholipid syndrome, and Sjögren's syndrome: evidence- and practice-based guidance.Frontiers in immunology · 2023Review
- A case report of acute heart failure and cardiogenic shock caused by catastrophic antiphospholipid syndrome and lupus myocarditis.European heart journal. Case reports · 2022Article
- Minimal role for the alternative pathway in complement activation by HIT immune complexes.Journal of thrombosis and haemostasis : JTH · 2022Article
- Emerging Therapies in Antiphospholipid Syndrome.Transfusion medicine reviews · 2022Review
- Eculizumab for refractory thrombosis in antiphospholipid syndrome.Blood advances · 2022Article
- Cases of catastrophic anti-phospholipid syndrome in systemic lupus erythematosus: An experience.SAGE open medical case reports · 2022Article
- Managing Antiphospholipid Syndrome in Children and Adolescents: Current and Future Prospects.Paediatric drugs · 2022Review
- Article
- Immunosuppressive Treatment in Antiphospholipid Syndrome: Is It Worth It?Biomedicines · 2021Review
- Complement in Secondary Thrombotic Microangiopathy.Kidney international reports · 2021Review
- A Case Report of COVID-Associated Catastrophic Antiphospholipid Syndrome Successfully Treated with Eculizumab.Journal of blood medicine · 2021Article
- Antiphospholipid syndrome.Translational research : the journal of laboratory and clinical medicine · 2020Review
Corrections and comments
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Authors and funding
7 authors at 3 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Catastrophic antiphospholipid syndrome (CAPS) is a rare disorder, characterized by the development of multiple vascular thrombosis over a short period of time, in patients with persistently detectable antiphospholipid antibodies (aPLs). The vascular occlusions predominantly affect small vessels. The overall mortality is 36.9%, despite the recent progress in the therapeutic approach. It has been shown that aPLs are able to induce a hypercoagulability state through different mechanisms of action, including complement activation, which in turn plays a key role in the pathogenesis of some thrombotic microangiopathies. Consequently, complement inhibition may be proposed as a targeted intervention to effectively prevent the progression of the microthrombotic storm. The employment of the complement inhibitor eculizumab has been proposed in CAPS on the basis of occasional reports and expert opinion. We report the case of a 54-year-old woman with a CAPS refractory to conventional therapies, who was successfully treated with eculizumab. The administration of this anti-C5 monoclonal antibody aborted the acute progressive thrombotic events and prevented further clinical episodes of thrombosis in the following year. We also faced our case to a systematic literature review, by analyzing all reported cases of CAPS in which eculizumab was added to conventional therapy. Even if further investigation is needed, our results suggest that the inhibition of one mechanism of aPL-induced organ damage may be an add-on treatment for this condition.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.