Treatment of antiphospholipid syndrome beyond anticoagulation

Dobrowolski, Chrisanna; Erkan, Doruk

doi:10.1016/j.clim.2018.03.001

Cited by 27 publications

(18 citation statements)

References 160 publications

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“…Since anticoagulation was recommended for all CTEPH patients as a life-long treatment, HCQ has been proven to be effective to prevent postoperative venous thrombotic events. [ 17 ] Other target treatments include statin therapy to reduce thrombotic risks and rituximab for refractory thrombosis.…”

Section: Discussionmentioning

confidence: 99%

Curative resolution of chronic thromboembolic pulmonary hypertension with pulmonary thromboendarterectomy in primary antiphospholipid syndrome

Li¹,

Zhao²,

et al. 2018

Medicine

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Rationale:Chronic thromboembolic pulmonary hypertension (CTEPH) is a severe complication of antiphospholipid syndrome (APS). Once diagnosed, the recommendation for the treatment of CTEPH is long-term anticoagulation and pulmonary thromboendarterectomy (PTE). However, cardiac surgeons apply PTE cautiously for these patients, as there is an increased risk of perioperative complications. Here, we present the curative case of a patient with severe APS-associated CTEPH treated with PTE.Patient concerns:A 29-year-old man presented with chest pain, decreased exercise capacity, dyspnoea, and haemoptysis.Diagnoses:He was triple positive for antiphospholipid antibodies. Computed tomography pulmonary angiography revealed multiple, recurrent pulmonary embolisms and complete obstruction of the left pulmonary artery. He was diagnosed with APS and CTEPH.Interventions:After balancing the risk of thrombosis and haemorrhage, the patient underwent PTE.Outcomes:The patient experienced symptom relief after PTE, and electrocardiography at a six-month follow-up showed a recovery of cardiac structure and pulmonary arterial pressure.Lessons:After evaluating the thrombosis risk at an experienced treatment centre and the application of standard anticoagulation treatment, PTE may be a curative resolution for APS-associated CTEPH.

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Section: Discussionmentioning

confidence: 99%

Curative resolution of chronic thromboembolic pulmonary hypertension with pulmonary thromboendarterectomy in primary antiphospholipid syndrome

Li¹,

Zhao²,

et al. 2018

Medicine

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“…Although anticoagulation therapy is part of the treatment regimen in some centers, there is no strong clinical support for its effectiveness, and many patients develop microvascular disease while receiving anticoagulation therapy. Given the increasing awareness of the mechanisms involved in APS pathogenesis (e.g., aPL‐induced endothelial cell, platelet, monocyte, neutrophil, complement, and coagulation system activation [12,13]), immunosuppressive therapies targeting different mechanisms are used with variable success in the management of microvascular disease in aPL‐positive patients (Tables 1 and 2).…”

Section: Clinical Challengementioning

confidence: 99%

“…Data on other complement inhibitors (e.g., ravulizumab) are lacking. complement, and coagulation system activation [12,13]), immunosuppressive therapies targeting different mechanisms are used with variable success in the management of microvascular disease in aPL-positive patients (Tables 1 and 2).…”

Section: Treatment Approach and Evidence (Maps)mentioning

confidence: 99%

Expert Perspective: Management of Microvascular and Catastrophic Antiphospholipid Syndrome

Erkan

2021

Arthritis & Rheumatology

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Clinical challengeA 50-year-old man with antiphospholipid syndrome (APS) presents to your office for the first time with a 3-month history of worsening dyspnea, dry cough, and early morning blood-tinged sputum. His APS diagnosis was based on an unprovoked left leg deep vein thrombosis, 4 years prior to the presentation, with persistent (multiple occasions) triple-antiphospholipid antibody (aPL) positivity, defined as lupus anticoagulant (LAC) positivity while not receiving anticoagulation therapy, high-titer (≥80 units) IgG anticardiolipin antibodies (aCL), and high-titer IgG anti-β 2 -glycoprotein I (anti-β 2 GPI). He has no other past medical history, and he is receiving warfarin with a target international normalized ratio (INR) of 2.5-3. Physical examination is normal except for livedo racemosa around his knees. His platelet count is 99 × 10 3 /ml (chronic; fluctuating 80-120 × 10 3 /ml since the APS diagnosis), INR is 3, hemoglobin, creatinine, and complement levels are normal, and spot urine protein:creatinine ratio is 0.3. His primary care physician ordered a chest radiograph, which shows extensive patchy bilateral airspace opacities, and a chest computed tomography (CT) scan, which shows diffuse bilateral ground-glass opacities.

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“…In treating thrombosis refractory to initial anticoagulation, the first treatment measure is to ensure that the patient has achieved a target INR and a therapeutic factor X level [ 74 ]. To prevent the development of recurrent thrombosis, the patient's INR should be 2.5-3.0, and they must be supplemented with low-dose aspirin if the patient has cardiovascular risk factors [ 75 ]. In addition, for patients diagnosed with recurrent thromboses and who are currently on optimal warfarin therapy, a higher INR target of 3.0-4.0 should be targeted with or without low-dose aspirin, hydroxychloroquine, or low-dose aspirin, hydroxychloroquine a statin [ 76 ].…”

Section: Reviewmentioning

confidence: 99%

Role of Direct Oral Anticoagulation Agents as Thromboprophylaxis in Antiphospholipid Syndrome

Arora

Nair²,

Prabhu³

et al. 2021

Cureus

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Antiphospholipid syndrome (APS) is an autoimmune disorder that causes venous, arterial and small-vessel thrombosis, pregnancy loss, and premature birth. Cardiac valvular disease, renal thrombotic microangiopathy, thrombocytopenia, hemolytic anemia, and cognitive impairment are some of its other clinical symptoms. Antiphospholipid antibodies cause endothelial cells, monocytes, and platelets to become activated, as well as an increase in tissue factor and thromboxane A2. Complement activation might play a key function in pathogenesis.Long-term oral anticoagulation is used to treat thrombosis, and individuals having arterial episodes should be treated quickly. Patients with systemic lupus erythematosus (SLE), as well as those with solely obstetric antiphospholipid syndrome, should get primary thromboprophylaxis. Obstetric care is based on a combination of medical and obstetric high-risk management, as well as aspirin and heparin therapy. Possible supplementary therapy for this condition is hydroxychloroquine. Statins, rituximab, and novel anticoagulant medicines are all potential future treatments for non-pregnant individuals with antiphospholipid syndrome. We aim to review the role of direct-acting oral anticoagulants (DOACs) as thromboprophylactic drugs in the treatment of APS in this article.The treatment of venous thromboembolism has been transformed by a new class of DOACs. These drugs, such as rivaroxaban, function by inhibiting factor Xa directly. Not only do they have known anticoagulant actions, but they also obviate the need for dosage monitoring and modification, in contrast to warfarin.We conducted an exhaustive literature search of PubMed/MEDLINE and Google Scholar Indexes using the keywords "Antiphospholipid syndrome," "thromboprophylaxis," and "oral anticoagulants" up to September 2021. We found that DOACs have been shown to be non-inferior to warfarin in a variety of anticoagulation situations in a number of high-powered clinical studies. In many hypercoagulable conditions such as APS, DOACs are quickly establishing themselves as first-line therapy. This article is focused on comprehensively reviewing the mechanism of action of DOACs, their role as thromboprophylactic drugs, risks and complications of DOACs, and comparing their efficacy with the standard treatment protocol and warfarin.

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Treatment of antiphospholipid syndrome beyond anticoagulation

Cited by 27 publications

References 160 publications

Curative resolution of chronic thromboembolic pulmonary hypertension with pulmonary thromboendarterectomy in primary antiphospholipid syndrome

Curative resolution of chronic thromboembolic pulmonary hypertension with pulmonary thromboendarterectomy in primary antiphospholipid syndrome

Expert Perspective: Management of Microvascular and Catastrophic Antiphospholipid Syndrome

Role of Direct Oral Anticoagulation Agents as Thromboprophylaxis in Antiphospholipid Syndrome

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