Eculizumab Induces Sustained Remission in a Patient With Refractory Primary Catastrophic Antiphospholipid Syndrome

Zikos, Thomas; Sokolove, Jeremy; Ahuja, Neera; Berubé, Caroline

doi:10.1097/rhu.0000000000000290

Cited by 68 publications

(34 citation statements)

References 12 publications

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“…There is evidence supporting activation of the classical and alternative complement pathways in patients with catastrophic APS, 109 and eculizumab (humanized anti-C5a monoclonal antibody) has been successfully used in CAPS and APS complicating renal transplantation. 110–114 …”

Section: Pathogenesis Of Apsmentioning

confidence: 99%

“…Eculizumab has been reported to successfully treat patients with refractory CAPS, 110–114 and an ongoing phase 2 study is evaluating the efficacy of eculizumab in preventing recurrent CAPS after renal transplantation (clinicaltrials.gov #NCT01029587). Eculizumab increases the risk of meningococcal infection and patients should be vaccinated before starting treatment.…”

Section: Treatment Of Catastrophic Apsmentioning

confidence: 99%

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Diagnosis and management of the antiphospholipid syndrome

2017

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Antiphospholipid syndrome (APS) is characterized by thrombosis and/or pregnancy complications in the presence of persistent antiphospholipid antibodies (APLA). Laboratory diagnosis of APLA depends upon the detection of a lupus anticoagulant, which prolongs phospholipid-dependent anticoagulation tests, and/or anticardiolipin (aCL) and anti-β2-glycoprotein-1 (β2GPI) antibodies. APLA are primarily directed towards phospholipid binding proteins. Pathophysiologic mechanisms underlying thrombosis and pregnancy loss in APS include APLA induced cellular activation, inhibition of natural anticoagulant and fibrinolytic systems, and complement activation, among others. There is a high rate of recurrent thrombosis in APS, especially in triple positive patients (patients with lupus anticoagulants, aCL and anti-β2GPI antibodies), and indefinite anticoagulation with a vitamin K antagonist is the standard of care for thrombotic APS. There is currently insufficient evidence to recommend the routine use of direct oral anticoagulants (DOAC) in thrombotic APS. Aspirin with low molecular weight or unfractionated heparin may reduce the incidence of pregnancy loss in obstetric APS. Recent insights into the pathogenesis of APS have led to the identification of new potential therapeutic interventions, including anti-inflammatory and immunomodulatory therapies. Additional research is needed to better understand the effects of APLA on activation of signaling pathways in vascular cells, to identify more predictive biomarkers that define patients at greatest risk for a first or recurrent APLA-related clinical event, and to determine the safety and efficacy of DOACs and novel anti-inflammatory and immune-modulatory therapies for refractory APS.

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Section: Pathogenesis Of Apsmentioning

confidence: 99%

Section: Treatment Of Catastrophic Apsmentioning

confidence: 99%

Diagnosis and management of the antiphospholipid syndrome

2017

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“…For example, APS is associated with poor outcomes following renal transplant, and two cases have been reported in which a CAPS-like picture was mitigated (and renal function preserved) with administration of eculizumab [62]*. Additionally, several case reports have suggested efficacy in CAPS patients who failed to respond to triple therapy, and sometimes also rituximab [63-66]. With caution, eculizumab may therefore be considered as a medication of last resort in CAPS, when organ- and life-threatening disease persists despite treatment with triple therapy.…”

Section: Therapymentioning

confidence: 99%

Treatment of catastrophic antiphospholipid syndrome

Kazzaz

McCune

Knight

2016

Current Opinion in Rheumatology

106

113

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Purpose of review Catastrophic antiphospholipid syndrome (CAPS) is a severe manifestation of APS. While affecting only 1% of patients with APS, the condition is frequently fatal if not recognized and treated early. Here, we will review the current approach to diagnosis and treatment of CAPS. Recent findings Data from the international “CAPS registry,” spearheaded by the European Forum on Antiphospholipid Antibodies, have improved our understanding of at-risk patients, typical clinical features, and associated/precipitating diagnoses. Current guidelines also continue to support a role for anticoagulants and glucocorticoids as foundation therapy in all patients. Finally, new basic science and case series suggest that novel therapies, such as rituximab and eculizumab warrant further study. Summary Attention to associated diagnoses such as infection and systemic lupus erythematosus (SLE) are critical at the time of diagnosis. All patients should be treated with anticoagulation, corticosteroids, and possibly plasma exchange. In patients with SLE, cyclophosphamide should also be considered. In refractory or relapsing cases, new therapies such as rituximab and possibly eculizumab may be options, but need further study.

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“…It is currently used in the treatment of paroxysmal nocturnal hemoglobinuria (PNH), atypical uremic syndrome (aHUS), generalized myasthenia gravis (gMG), and neuromyelitis optica spectrum disorder (NMOSD) [13]. There have been case reports showing the success of eculizumab in the treatment of refractory CAPS [14][15][16][17][18][19]. Our patient was started on an induction dose of 600 mg with undetectable complement levels which suggested appropriate medication activity.…”

Section: Discussionmentioning

confidence: 91%

Therapeutic Challenges with Catastrophic Antiphospholipid Antibody Syndrome: A Case Report

Harne

Sampat

Bisen

et al. 2020

Cureus

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Antiphospholipid antibody syndrome (APS) is a multisystem disorder characterized by thromboembolic events in the presence of antiphospholipid antibodies (APLA). Catastrophic antiphospholipid antibody syndrome (CAPS) is an uncommon variant of APS which is associated with widespread coagulopathy that predominantly affects small vessels. Despite maximal treatment, CAPS has a very high mortality rate. We present a case of a 42-year-old woman with a history of APS who presented to our hospital with complaints of epistaxis, hemoptysis, menorrhagia, and shortness of breath. She was diagnosed with CAPS and developed multiorgan failure and sepsis. Despite maximal treatment with immune modulators, she unfortunately succumbed. With this case, we highlight the importance of early recognition of CAPS and review various treatment modalities that have been proven beneficial. Despite these modalities, CAPS remains a therapeutic challenge in many cases and has a high mortality rate.

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Eculizumab Induces Sustained Remission in a Patient With Refractory Primary Catastrophic Antiphospholipid Syndrome

Cited by 68 publications

References 12 publications

Diagnosis and management of the antiphospholipid syndrome

Diagnosis and management of the antiphospholipid syndrome

Treatment of catastrophic antiphospholipid syndrome

Therapeutic Challenges with Catastrophic Antiphospholipid Antibody Syndrome: A Case Report

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