An update on the management of antiphospholipid syndrome

Rodziewicz, Mia; D’Cruz, David

doi:10.1177/1759720x20910855

Cited by 42 publications

(47 citation statements)

References 60 publications

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“…APS can occur idiopathically or it can be associated with other autoimmune diseases such as systemic lupus erythematosus ( 26 ). Catastrophic antiphospholipid syndrome (CAPS) is a severe manifestation of APS ( 26 ). Although affecting only 1% of patients with APS, the condition is frequently fatal if not recognized and treated early.…”

Section: Can the Thromboembolic Diathesis During Some Cases Of Covmentioning

confidence: 99%

“…In addition, several anecdotic reports have proposed beneficial effects of immunomodulatory agents including corticosteroids, rituximab, IvIg, D3 vitamin, plasmapheresis and chloroquine, anti-complement antibody eculizumab and the mTOR inhibitor rapamycin ( 26 , 40 , 41 ).…”

Section: Can the Thromboembolic Diathesis During Some Cases Of Covmentioning

confidence: 99%

“…In a similar manner, future studies are required for those individuals with single, dual or triple positivity for LA and aPL Abs that are clinically asymptomatic to prove whether they are at increased risks of thromboembolism in case of COVID-19 infection. Although thromboprophylaxis is currently not approved for these individuals ( 26 ) this therapeutic approach may need to be revised during COVID-19 pandemic. The testing of large cohort of COVID-19 patients for aPL Abs is also of outmost relevance to understand the real prevalence of these autoantibodies in patients with SARS-CoV-2.…”

Section: Can the Thromboembolic Diathesis During Some Cases Of Covmentioning

confidence: 99%

“…The anti-C5a mAb eculizumab would also receive attention though its costs would hinder its repurposing in a very large arena such as is that of COVID-19 currently. The use of plasmapheresis should warrant further consideration as well as that of IvIg that seem effective in some cases of APS and CAPS ( 26 , 40 ). Small molecule inhibitors of Toll-like receptor 4 signaling such as resatorvid or GLS-1027 R that may also inhibit neutrophil extracellular traps associated with thrombosis, might be considered in the therapeutic management of COVID-19-related thrombosis ( 58 , 59 ).…”

Section: Future Therapeutic Directionsmentioning

confidence: 99%

“…Background. The APS is characterized by the occurrence of multiple episodes of venous and arterial thromboses and recurrent fetal losses, frequently accompanied by a moderate thrombocytopenia, in the presence of antiphospholipid (auto) antibodies (aPL Abs) that are directed against cardiolipin (acL) or β2 glycoprotein1 (β2-gP1) (26). Activation of the complement is also required for the full clinical manifestation of the APS.…”

Section: Can the Thromboembolic Diathesis During Some Cases Of Covid-19 Represents Secondary Form Of Anti-phospholipid Antibody Syndrome?mentioning

confidence: 99%

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Entangling COVID-19 associated thrombosis into a secondary antiphospholipid antibody syndrome: Diagnostic and therapeutic perspectives (Review)

et al. 2020

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The severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) is a novel β coronavirus that is the etiological agent of the pandemic coronavirus disease 2019 (COVID-19) that at the time of writing (June 16, 2020) has infected almost 6 million people with some 450,000 deaths. These numbers are still rising daily. Most (some 80%) cases of COVID-19 infection are asymptomatic, a substantial number of cases (15%) require hospitalization and an additional fraction of patients (5%) need recovery in intensive care units. Mortality for COVID-19 infection appears to occur globally between 0.1 and 0.5% of infected patients although the frequency of lethality is significantly augmented in the elderly and in patients with other comorbidities. The development of acute respiratory distress syndrome and episodes of thromboembolism that may lead to disseminated intravascular coagulation (DIC) represent the primary causes of lethality during COVID-19 infection. Increasing evidence suggests that thrombotic diathesis is due to multiple derangements of the coagulation system including marked elevation of D-dimer that correlate negatively with survival. We propose here that the thromboembolic events and eventually the development of DIC provoked by SARS-CoV-2 infection may represent a secondary anti-phospholipid antibody syndrome (APS). We will apply both Baconian inductivism and Cartesian deductivism to prove that secondary APS is likely responsible for coagulopathy during the course of COVID-19 infection. Diagnostic and therapeutic implications of this are also discussed.

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Section: Can the Thromboembolic Diathesis During Some Cases Of Covmentioning

confidence: 99%

Section: Can the Thromboembolic Diathesis During Some Cases Of Covmentioning

confidence: 99%

Section: Can the Thromboembolic Diathesis During Some Cases Of Covmentioning

confidence: 99%

Section: Future Therapeutic Directionsmentioning

confidence: 99%

Section: Can the Thromboembolic Diathesis During Some Cases Of Covid-19 Represents Secondary Form Of Anti-phospholipid Antibody Syndrome?mentioning

confidence: 99%

See 3 more Smart Citations

Entangling COVID-19 associated thrombosis into a secondary antiphospholipid antibody syndrome: Diagnostic and therapeutic perspectives (Review)

et al. 2020

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Vitamin K Antagonist Reversal for Urgent Surgery Using 4-Factor Prothrombin Complex Concentrates

Sarode,

Goldstein,

Simonian

et al. 2024

JAMA Netw Open

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ImportanceMillions of people take vitamin K antagonists (VKAs). Some people who need urgent surgical procedures require rapid VKA reversal to prevent excessive intraoperative bleeding.ObjectiveTo evaluate the hemostatic noninferiority of an investigational 4-factor prothrombin complex concentrate (4F-PCC) to a control 4F-PCC for rapid VKA reversal before urgent surgery.Design, Setting, and ParticipantsThis phase 3, double-blind, noninferiority randomized clinical trial (LEX-209) was conducted in 24 hospitals in the US, Russia, Georgia, Belarus, Ukraine, and Romania from June 7, 2017, through November 8, 2021; the study was stopped in February 2022. Participants were adult patients taking VKA who had an international normalized ratio (INR) of 2 or higher and needed urgent surgery with a substantial bleeding risk (≥50 mL). Patients were randomized 1:1 to a single infusion of either the investigational 4F-PCC or the control 4F-PCC. Data analysis followed intention-to-treat and per-protocol approaches.InterventionsSingle intravenous infusion was dosed by body weight and baseline INR. A dose of 25, 35, or 50 IU/kg of investigational 4F-PCC or control 4F-PCC was administered for baseline INR of 2 to less than 4, 4 to 6, or over 6, respectively.Main Outcome and MeasureThe primary end point was hemostatic efficacy at surgery end. An independent adjudication board, blinded to the 4F-PCC treatment allocation, assessed hemostatic efficacy using an objective 4-point scale.ResultsA total of 208 patients (median [range] age, 67.5 [31-92] years; 118 males [56.7%]) received the investigational (n = 105) or the control (n = 103) 4F-PCC. The median (range) dose was 25 (16-50) IU/kg in the investigational group and 25 (15-50) IU/kg in the control group, with a median (range) infusion time of 12 (8-50) minutes and 13 (7-30) minutes and a median (range) time from infusion to surgery start of 1.42 (0.25-15.25) hours and 1.50 (0.42-18.50) hours, respectively. Baseline median (range) INR was 3.05 (1.97-21.10) in the investigational group and 3.00 (2.00-11.30) in the control group. In the intention-to-treat analysis, the investigational 4F-PCC was noninferior to the control 4F-PCC, resulting in effective hemostasis in 94.3% of patients vs 94.2% of patients (proportion difference, 0.001; 95% CI, −0.080 to 0.082; P &lt; .001), meeting the prespecified noninferiority margin of 0.15. An INR of 1.5 or lower at 30 minutes after infusion occurred in 78.1% of patients in the investigational group vs 71.8% of patients in the control group (proportion difference, 0.063; 95% CI, −0.056 to 0.181). Thrombotic events (2.9% vs 0%, respectively) and mortality (4.8% vs 1.0%, respectively) were no different than expected for 4F-PCC use. One patient in each treatment group discontinued due to adverse events (cardiac disorders unrelated to 4F-PCC).Conclusions and RelevanceThis randomized clinical trial found that the investigational 4F-PCC was hemostatically noninferior to the control 4F-PCC for rapid VKA reversal in patients needing urgent surgery with considerable bleeding risk; the safety profile of these two 4F-PCCs was similar. These results support the investigational 4F-PCC as a therapeutic option for surgical patients requiring rapid VKA reversal.Trial RegistrationClinicalTrials.gov Identifier: NCT02740335

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Severe cutaneous necrosis in antiphospholipid syndrome

Mostafa,

Mahmoud,

Egiza

et al. 2023

American J Hematol

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Antiphospholipid syndrome (APS) is a systemic autoimmune disorder characterized by recurrent thrombosis and/or recurrent fetal loss. APS can be primary or secondary to autoimmune disorders such as systemic lupus erythematosus (SLE). It is unique from other thrombophilic disorders in that it predisposes patients to not only venous but also arterial thrombosis through endothelial cell damage and activation of platelets. 1 The Sydney Criteria are used for diagnosis of APS and necessitate the presence of one clinical and one laboratory criteria. 2 Clinical criteria consist of objectively confirmed venous, arterial or small vessel thrombosis, or pregnancy complications that may be attributed to placental insufficiency, including pre-eclampsia, pregnancy loss, or premature birth. Laboratory criteria consist of a positive laboratory test (lupus anticoagulant, anticardiolipin anticoagulant and/or anti-B2 glycoprotein) found on 2 or more occasions at least 12 weeks apart. 2 Management of APS involves the long-term use of vitamin K antagonists.Catastrophic APS (CAPS), as the name implies, is a disastrous complication of APS. The International Congress on aPL has proposed

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An update on the management of antiphospholipid syndrome

Cited by 42 publications

References 60 publications

Entangling COVID-19 associated thrombosis into a secondary antiphospholipid antibody syndrome: Diagnostic and therapeutic perspectives (Review)

Entangling COVID-19 associated thrombosis into a secondary antiphospholipid antibody syndrome: Diagnostic and therapeutic perspectives (Review)

Vitamin K Antagonist Reversal for Urgent Surgery Using 4-Factor Prothrombin Complex Concentrates

Severe cutaneous necrosis in antiphospholipid syndrome

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