Materiały źródłowe

• #1

  https://www.nhs.uk/conditions/antiphospholipid-syndrome/

  Although there’s no cure for APS, the risk of developing blood clots can be greatly reduced if it’s correctly diagnosed. […] An anticoagulant medicine, such as warfarin, or an antiplatelet, such as low-dose aspirin, is usually prescribed. […] These reduce the likelihood of unnecessary blood clots forming, but still allow clots to form when you cut yourself. […] Treatment with these medications can also improve a pregnant woman’s chance of having a successful pregnancy. […] With treatment, it’s estimated there’s about an 80% chance of having a successful pregnancy. […] Most people respond well to treatment and can lead normal, healthy lives. […] But a small number of people with APS continue to experience blood clots despite extensive treatment.

• #2 Antiphospholipid Syndrome: Symptoms, Diagnosis and Treatments

  https://www.webmd.com/dvt/what-is-antiphospholipid-syndrome

  Scientists aren’t sure exactly what causes APS, which is most common in young women (and those assigned female at birth). There’s no cure, but treatment can help prevent blood clots from forming. […] Treatment for APS can reduce your risk for these complications. […] There’s no cure for antiphospholipid syndrome, but treatment can protect against complications. The goal of treatment is to stop new blood clots from forming and keep any you already have from growing larger. […] A combination of blood thinning drugs (anticoagulants) is the standard treatment. Most people take fast-heparin as a shot along with a slower-acting blood thinner in pill form called warfarin (Jantoven). Low-dose aspirin is another blood thinner. […] Treatment helps prevent miscarriages and other pregnancy complications of APS. During pregnancy, you can get injections of heparin up until just before delivery. You may also take low doses of aspirin. But warfarin isn’t safe during pregnancy. […] If you’ve had repeated miscarriages, your doctor may treat you with: Enoxaparin, an anticoagulant you get in a shot; Corticosteroids, which suppress an overactive immune system; Immunoglobulin, another drug used to treat immune system disorders, which you get through an IV.

• #3 An update on the biologics for the treatment of antiphospholipid syndrome

  https://pmc.ncbi.nlm.nih.gov/articles/PMC10237350/

  Antiphospholipid syndrome (APS) is a systemic autoimmune disease characterized by thrombosis and pregnancy morbidity with the persistent presence of antiphospholipid antibodies (aPLs). Although anticoagulation is the primary treatment for APS, it fails in approximately 20-30% of obstetric APS cases and more than 30% of thrombotic APS cases. Therefore, there is a need for new, targeted treatments beyond anticoagulants. Biologics, such as rituximab and eculizumab, have been recommended for refractory catastrophic APS. […] Anticoagulation therapy is considered a fundamental cornerstone of APS management. However, conventional prevention and treatment strategies fail in approximately 20-30% of obstetric APS and more than 30% of thrombotic APS cases. Furthermore, traditional medications are often ineffective in treating CAPS, refractory APS, and noncriteria manifestations.

• #4 Antiphospholipid Syndrome Treatment & Management: Medical Care, Surgical Care, Consultations

  https://emedicine.medscape.com/article/333221-treatment

  Patients with antiphospholipid syndrome (APS) may be evaluated in an outpatient setting. Inpatient evaluation is required if the patient presents with a significant clinical event. Patients with catastrophic APS (CAPS) require intense observation and treatment, often in an intensive care unit. […] In general, treatment regimens for APS must be individualized according to the patient’s current clinical status and history of thrombotic events. Asymptomatic individuals in whom blood test findings are positive do not require specific treatment. […] Prophylactic anticoagulation is needed during surgery or hospitalization. Management of any associated autoimmune disease is necessary. […] In patients who have antiphospholipid antibodies but no history of thrombosis or pregnancy morbidity, antithrombotic therapy is not routinely used as primary thrombosis prevention. However, low-dose aspirin is used in this setting; however, the effectiveness of low-dose aspirin as primary prevention for APS remains unproven.

• #5 EULAR recommendations for the management of antiphospholipid syndrome in adults | Annals of the Rheumatic Diseases

  https://ard.bmj.com/content/78/10/1296

  The objective was to develop evidence-based recommendations for the management of antiphospholipid syndrome (APS) in adults. […] Low-dose aspirin (LDA) is recommended for asymptomatic aPL carriers, patients with systemic lupus erythematosus without prior thrombotic or obstetric APS, and non-pregnant women with a history of obstetric APS only, all with high-risk aPL profiles. […] Patients with APS and first unprovoked venous thrombosis should receive long-term treatment with vitamin K antagonists (VKA) with a target international normalised ratio (INR) of 2-3. […] In patients with APS with first arterial thrombosis, treatment with VKA with INR 2-3 or INR 3-4 is recommended, considering the individuals bleeding/thrombosis risk. […] Rivaroxaban should not be used in patients with APS with triple aPL positivity.

• #6 Antiphospholipid Syndrome (APS) Diagnostics

  https://www.diagnostic.grifols.com/en/-/learning/antiphospholipid-syndrome-aps-diagnostics

  For those with APS, whether treatment is required will depend on the aPL profile, symptoms, and clinical profile. Generally, treatment is prophylactic to prevent thrombosis, and will continue in the long term, perhaps for life. […] From a review of published literature, the European League Against Rheumatism (EULAR) has developed a series of recommendations for the management of APS. Patients can be classified into risk categories according to aPL profile. High risk is the presence, on 2 occasions 12 weeks apart, of: LA, a double combination of any of LA, aCL antibody or aB2GP1 antibody, the triple combination of LA, aCL antibody and aB2GP1 antibody, persistently high aPL titers. The high-risk profile is associated with elevated risk of thrombosis and obstetric APS. […] Prophylactic low-dose aspirin is recommended for those with a high-risk aPL profile but no symptoms, as well as in those with SLE with no history of APS symptoms. Depending on risk/benefit analysis, it may also be prescribed for non-pregnant women with a history of obstetric APS. For these types of patients, this daily treatment may be required for life. In patients with definite APS and a first venous thrombosis, the recommended treatment (after heparin therapy) is a vitamin K antagonist (VKA) most commonly warfarin with a target international normalized ratio (INR) of 2-3. In those with definite APS and first arterial thrombosis, VKA is recommended over low-dose aspirin but, depending on the bleeding and recurrent thrombosis risk, low-dose aspirin may be added. If there is recurrent thrombosis (arterial or venous), low-dose aspirin can be added, the INR for anticoagulation can be increased to 3-4 or the anticoagulation can be switched to LMWH.

• #7 Anticoagulant Therapy in Patients with Antiphospholipid Syndrome

  https://www.mdpi.com/2077-0383/11/23/6984

  […] […] According to the most recent guidelines, primary antithrombotic prophylaxis with low dose (75–100 mg/day) aspirin (LDA) in high-risk profile aPLA patients without history of thrombosis is recommended. A metanalysis of 460 patients showed that those on LDA had a two-fold risk reduction of first thrombotic events than those without. However, the meta-analysis included mainly observational studies and the level of evidence for this recommendation is low. On the other hand, the use of low molecular weight heparin (LMWH) in high-risk situations such as postoperative periods, lower limb fracture, immobilization, hospitalization, pregnancy/puerperium or central venous catheter placement is widely accepted, as for all other severe thrombophilia abnormalities. […] […] […] For patients with APS and VTE, current guidelines recommend treatment with unfractionated heparin or LMWH followed by long-term vitamin K antagonists (VKA) with a target INR of 2.0 to 3.0. However, there is no consensus on the best strategy in terms of intensity of anticoagulation in different presentations of the disease and the usefulness of antiplatelet drugs, both in venous and arterial thrombosis.

• #8 EULAR recommendations for the management of antiphospholipid syndrome in adults | Annals of the Rheumatic Diseases

  https://ard.bmj.com/content/78/10/1296

  The objective was to develop evidence-based recommendations for the management of antiphospholipid syndrome (APS) in adults. […] Low-dose aspirin (LDA) is recommended for asymptomatic aPL carriers, patients with systemic lupus erythematosus without prior thrombotic or obstetric APS, and non-pregnant women with a history of obstetric APS only, all with high-risk aPL profiles. […] Patients with APS and first unprovoked venous thrombosis should receive long-term treatment with vitamin K antagonists (VKA) with a target international normalised ratio (INR) of 2-3. […] In patients with APS with first arterial thrombosis, treatment with VKA with INR 2-3 or INR 3-4 is recommended, considering the individuals bleeding/thrombosis risk. […] Rivaroxaban should not be used in patients with APS with triple aPL positivity.

• #9 An update on the management of antiphospholipid syndrome

  https://pmc.ncbi.nlm.nih.gov/articles/PMC7236388/

  VKAs are teratogenic; therefore, in APS patients with prior thrombosis or pregnancy morbidity, therapeutic dose low-molecular-weight heparin (LMWH) and LDA is accepted treatment. For those patients with purely obstetric APS and no prior thrombosis (OAPS), prophylactic dose LMWH and LDA until 6 weeks postpartum is recommended. […] The 13th International Congress on Antiphospholipid Antibodies task force, as well as current EULAR guidance recommend that patients with definite APS and a first venous event receive lifelong oral anticoagulation to a target INR of 2.0-3.0. […] Direct oral anticoagulants (DOACs) such as rivaroxaban, apixaban and dabigatran are licensed for use in the general population for the secondary prevention of VTE and the prevention of arterial thrombosis in nonvalvular atrial fibrillation.

• #10 Patient education: Antiphospholipid syndrome (Beyond the Basics) – UpToDate

  https://www.uptodate.com/contents/antiphospholipid-syndrome-beyond-the-basics

  TREATMENT AND PREVENTION OF BLOOD CLOTS […] Anticoagulants — Anticoagulants are medications that prevent an existing blood clot from enlarging and that prevent the formation of additional blood clots. Anticoagulants are commonly referred to as blood thinners, but they do not actually thin the blood. […] People with antiphospholipid syndrome (APS) and acute blood clots are often treated with an injectable anticoagulant called heparin or low molecular weight heparin (LMWH). In some cases, the heparin is given into a vein while the person is in the hospital. In other cases, heparin is injected under the skin. […] There are several types of LMWH, including enoxaparin (brand name: Lovenox), dalteparin (brand name: Fragmin), or tinzaparin (brand name: Innohep). These can be injected beneath the skin by the person, a family member, or a home health nurse. This is usually continued for several days, along with another medication called warfarin (brand name: Jantoven). Warfarin is an anticoagulant medication that is taken by mouth, as a pill, for the long-term treatment of blood clots and the prevention of recurrence. Heparin can be stopped once warfarin levels are stable.

• #11 Patient education: Antiphospholipid syndrome (Beyond the Basics) – UpToDate

  https://www.uptodate.com/contents/antiphospholipid-syndrome-beyond-the-basics

  TREATMENT AND PREVENTION OF BLOOD CLOTS […] Anticoagulants — Anticoagulants are medications that prevent an existing blood clot from enlarging and that prevent the formation of additional blood clots. Anticoagulants are commonly referred to as blood thinners, but they do not actually thin the blood. […] People with antiphospholipid syndrome (APS) and acute blood clots are often treated with an injectable anticoagulant called heparin or low molecular weight heparin (LMWH). In some cases, the heparin is given into a vein while the person is in the hospital. In other cases, heparin is injected under the skin. […] There are several types of LMWH, including enoxaparin (brand name: Lovenox), dalteparin (brand name: Fragmin), or tinzaparin (brand name: Innohep). These can be injected beneath the skin by the person, a family member, or a home health nurse. This is usually continued for several days, along with another medication called warfarin (brand name: Jantoven). Warfarin is an anticoagulant medication that is taken by mouth, as a pill, for the long-term treatment of blood clots and the prevention of recurrence. Heparin can be stopped once warfarin levels are stable.

• #12 Antiphospholipid Syndrome Treatment & Management: Medical Care, Surgical Care, Consultations

  https://emedicine.medscape.com/article/333221-treatment

  Clopidogrel has anecdotally been reported to be helpful in persons with APS and may be useful in patients allergic to aspirin. […] Perform full anticoagulation with intravenous or subcutaneous heparin followed by warfarin therapy. Based on the most recent evidence, a reasonable target for the international normalized ratio (INR) is 2.0-3.0 for venous thrombosis and 3.0 for arterial thrombosis. Patients with recurrent thrombotic events may require an INR of 3.0-4.0. For severe or refractory cases, a combination of warfarin and aspirin may be used. Treatment for significant thrombotic events in patients with APS is generally lifelong. […] Direct oral anticoagulants (ie, direct thrombin inhibitors and factor Xa inhibitors such as rivaroxaban) have been used in patients with warfarin intolerance/allergy or poor anticoagulant control.

• #13 Antiphospholipid Syndrome and Its Management

  https://www.uspharmacist.com/article/antiphospholipid-syndrome-and-its-management

  In patients with APS defined by venous thrombosis, it is recommended to initially treat with unfractionated heparin or low-molecular-weight heparin (LMWH) followed by long-term anticoagulation with a VKA such as warfarin. […] Long-term anticoagulation is recommended in most patients who have persistent aPLs and who had an otherwise unprovoked VTE. However, the benefit of prolonged anticoagulation is less clear in patients who are positive for aPLs but had a provoked thrombus and in patients whose laboratory tests for aPLs become negative over time. […] There is less consensus about the optimal management of APS patients who have experienced an arterial event. Patients with moderate-risk to high-risk aPL profiles are often treated with warfarin (INR goal, 2-3). Some clinicians prefer higher-intensity warfarin therapy (INR goal, 3-4) for patients with arterial thrombosis, based on the paucity of data on patients with arterial thrombosis in randomized, controlled trials comparing different intensities of warfarin therapy.

• #14 EULAR recommendations for the management of antiphospholipid syndrome in adults | Annals of the Rheumatic Diseases

  https://ard.bmj.com/content/78/10/1296

  The objective was to develop evidence-based recommendations for the management of antiphospholipid syndrome (APS) in adults. […] Low-dose aspirin (LDA) is recommended for asymptomatic aPL carriers, patients with systemic lupus erythematosus without prior thrombotic or obstetric APS, and non-pregnant women with a history of obstetric APS only, all with high-risk aPL profiles. […] Patients with APS and first unprovoked venous thrombosis should receive long-term treatment with vitamin K antagonists (VKA) with a target international normalised ratio (INR) of 2-3. […] In patients with APS with first arterial thrombosis, treatment with VKA with INR 2-3 or INR 3-4 is recommended, considering the individuals bleeding/thrombosis risk. […] Rivaroxaban should not be used in patients with APS with triple aPL positivity.

• #15 Antiphospholipid Syndrome and Its Management

  https://www.uspharmacist.com/article/antiphospholipid-syndrome-and-its-management

  In patients with APS defined by venous thrombosis, it is recommended to initially treat with unfractionated heparin or low-molecular-weight heparin (LMWH) followed by long-term anticoagulation with a VKA such as warfarin. […] Long-term anticoagulation is recommended in most patients who have persistent aPLs and who had an otherwise unprovoked VTE. However, the benefit of prolonged anticoagulation is less clear in patients who are positive for aPLs but had a provoked thrombus and in patients whose laboratory tests for aPLs become negative over time. […] There is less consensus about the optimal management of APS patients who have experienced an arterial event. Patients with moderate-risk to high-risk aPL profiles are often treated with warfarin (INR goal, 2-3). Some clinicians prefer higher-intensity warfarin therapy (INR goal, 3-4) for patients with arterial thrombosis, based on the paucity of data on patients with arterial thrombosis in randomized, controlled trials comparing different intensities of warfarin therapy.

• #16 Antiphospholipid Syndrome and Its Management

  https://www.uspharmacist.com/article/antiphospholipid-syndrome-and-its-management

  The 16th International Congress on Antiphospholipid Antibodies report found insufficient evidence for strong recommendations concerning the use of LDA for secondary prevention following a first APS-related arterial thrombosis; however, it stated that LDA use could be considered in combination with standard-intensity VKA therapy (INR goal, 2-3). […] There is limited high-quality evidence supporting any management strategy when the use of warfarin fails despite the presence of a therapeutic INR. Options include higher-intensity warfarin therapy (target INR, 3-4); the addition of LDA, hydroxychloroquine (HCQ), or a statin; the use of a different anticoagulant; or some combination of these approaches. […] According to the 16th International Congress on Antiphospholipid Antibodies report, LDA may be considered in combination with anticoagulation in patients who develop recurrent arterial or venous thrombosis on a standard-intensity VKA.

• #17 Antiphospholipid Syndrome and Stroke – European Stroke Organisation

  https://eso-stroke.org/antiphospholipid-syndrome-and-stroke/

  As the APS varies in its phenotype and severity, an individual treatment approach is necessary. Primary prevention with antiplatelets therapy should be started only in patients with additional risk factors, including cardiovascular comorbidities, eventually high-risk APS profile. […] However, in stroke patients with positive aPL antibodies not fulfilling diagnostic criteria for APS a similar approach as in stroke patients with negative aPL is recommended. For patients who meet the criteria for APS, moderate-intensity anticoagulation (INR 2-3) is reasonable. […] High-intensity anticoagulation (INR 3.1) or heparinoids are often used in clinical practice if patients suffer a recurrent thromboembolic event despite moderate-intensity anticoagulation. […] Another approach may be moderate-intensity anticoagulation combined with antiplatelet which may, however, lead to higher bleeding risk. […] There are scarce data on the usage of direct oral anticoagulants (DOAC) in APS-related stroke. […] Here, a high-dose steroid treatment, i.v. immunoglobulins or plasma exchange alongside intensive anticoagulation treatment is often indicated.

• #18 EULAR recommendations for the management of antiphospholipid syndrome in adults | Annals of the Rheumatic Diseases

  https://ard.bmj.com/content/78/10/1296

  For patients with recurrent arterial or venous thrombosis despite adequate treatment, addition of LDA, increase of INR target to 3-4 or switch to low molecular weight heparin may be considered. […] In women with prior obstetric APS, combination treatment with LDA and prophylactic dosage heparin during pregnancy is recommended. […] In patients with recurrent pregnancy complications, increase of heparin to therapeutic dose, addition of hydroxychloroquine or addition of low-dose prednisolone in the first trimester may be considered. […] These recommendations aim to guide treatment in adults with APS. High-quality evidence is limited, indicating a need for more research.

• #19 Antiphospholipid Syndrome and Its Management

  https://www.uspharmacist.com/article/antiphospholipid-syndrome-and-its-management

  The 16th International Congress on Antiphospholipid Antibodies report found insufficient evidence for strong recommendations concerning the use of LDA for secondary prevention following a first APS-related arterial thrombosis; however, it stated that LDA use could be considered in combination with standard-intensity VKA therapy (INR goal, 2-3). […] There is limited high-quality evidence supporting any management strategy when the use of warfarin fails despite the presence of a therapeutic INR. Options include higher-intensity warfarin therapy (target INR, 3-4); the addition of LDA, hydroxychloroquine (HCQ), or a statin; the use of a different anticoagulant; or some combination of these approaches. […] According to the 16th International Congress on Antiphospholipid Antibodies report, LDA may be considered in combination with anticoagulation in patients who develop recurrent arterial or venous thrombosis on a standard-intensity VKA.

• #20 Current concepts in the diagnosis and management of antiphospholipid syndrome and ocular manifestations | Journal of Ophthalmic Inflammation and Infection | Full Text

  https://joii-journal.springeropen.com/articles/10.1186/s12348-021-00240-8

  Statins, also known as hydroxymethylglutaryl coenzyme A (HMG-CoA) reductase inhibitors, are a type of cholesterol lowering agent. In addition to a lipid-lowering effect, statins have demonstrated anti-thrombotic and anti-inflammatory properties such as inhibition of tissue factor production in endothelial cells and prevention of anti-2GPI antibody-mediated endothelial adherence. Fluvastatin treatment has been proposed to decrease proinflammatory and prothrombotic mediators in aPL positive patients and to inhibit several mediators in monocytes that may be pro-thrombotic in APS patients. […] Rituximab is a chimeric monoclonal antibody against CD 20 expressed on B cells that was originally developed for the treatment of non-Hodgkins B-cell lymphoma. In the last several years, B-cell targeting therapies have been approved for the treatment of various autoimmune diseases. Anti-CD20 monoclonal therapy has been shown to decrease aPL titers and prevent new thrombotic events. Moreover, in a non-randomized prospective pilot study, rituximab was found to be effective in the treatment of non-criteria aPL manifestations in persistently aPL positive patients. It has also been a safe and effective therapeutic option for patients with refractory catastrophic APS.

• #21 Lupus anticoagulants and antiphospholipid antibodies Information | Mount Sinai – New York

  https://www.mountsinai.org/health-library/diseases-conditions/lupus-anticoagulants-and-antiphospholipid-antibodies

  The treatment for APS is directed at preventing complications from new blood clots forming or existing clots getting larger. You will need to take some form of blood-thinning medicine. If you also have an autoimmune disease, such as lupus, you will need to keep that condition under control as well. […] In general, you will need treatment with a blood thinner for a long time if you have APS. Initial treatment may be heparin. This medicine is given by injection. […] In most cases, warfarin (Coumadin), which is given by mouth, is then started. It is necessary to frequently monitor the level of anticoagulation. This is most often done using the INR test. […] If you have APS and become pregnant, you will need to be followed closely by a provider who is an expert in this condition. You will not take warfarin during pregnancy, but will be given heparin shots instead.

• #22 Patient education: Antiphospholipid syndrome (Beyond the Basics) – UpToDate

  https://www.uptodate.com/contents/antiphospholipid-syndrome-beyond-the-basics

  Heparin is usually started once pregnancy is confirmed and is usually stopped temporarily for planned procedures (eg, amniocentesis, cesarean section) and when labor begins. Either heparin or warfarin is recommended for six to eight weeks after delivery. […] Warfarin — Warfarin is not recommended for pregnant people with APS because of the potential risks to the developing fetus. Warfarin must be stopped before six weeks of pregnancy. […] Warfarin can be restarted after delivery and is often the preferred treatment for people with APS during the postpartum stage (the six to eight weeks following delivery). Warfarin is safe to take while breastfeeding. […] Preventing pregnancy — People with aPL (with or without APS) should not use birth control methods that contain estrogen because estrogen can increase the risk of developing a blood clot. A number of other methods of birth control are available, including progestin-only pills, an intrauterine device (IUD), condoms, a diaphragm, an injection (eg, Depo Provera), or a surgical procedure.

• #23

  https://www.nhs.uk/conditions/antiphospholipid-syndrome/treatment/

  Women diagnosed with APS are strongly advised to plan for any future pregnancy. […] Treatment during pregnancy involves taking daily doses of aspirin or heparin, or a combination of both. […] Warfarin isn’t recommended during pregnancy because it carries a small risk of causing birth defects. […] Treatment with aspirin or heparin, or both, is usually started at the beginning of the pregnancy and may continue for 1 to 6 weeks after you have given birth.

• #24 Diagnosis and management of antiphospholipid syndrome – Australian Prescriber

  https://australianprescriber.tg.org.au/articles/diagnosis-and-management-of-antiphospholipid-syndrome.html

  If a patient with a history of obstetric manifestations (but no history of thrombotic manifestations) becomes pregnant, both prophylactic-dose low-molecular-weight heparin and low-dose aspirin are recommended for the duration of the pregnancy and postpartum for 6 to 12 weeks. […] If a patient with a history of thrombotic manifestations (but no history of obstetric manifestations) becomes pregnant, therapeutic-dose low-molecular-weight heparin and low-dose aspirin are recommended for the duration of the pregnancy and for 6 to 12 weeks postpartum. […] If catastrophic APS is suspected, the patient should be immediately referred to a hospital for assessment and management. Initial treatment involves anticoagulation and high-dose corticosteroids, and is often followed by plasma exchange and/or rituximab or cyclophosphamide.

• #25 EULAR recommendations for the management of antiphospholipid syndrome in adults | Annals of the Rheumatic Diseases

  https://ard.bmj.com/content/78/10/1296

  For patients with recurrent arterial or venous thrombosis despite adequate treatment, addition of LDA, increase of INR target to 3-4 or switch to low molecular weight heparin may be considered. […] In women with prior obstetric APS, combination treatment with LDA and prophylactic dosage heparin during pregnancy is recommended. […] In patients with recurrent pregnancy complications, increase of heparin to therapeutic dose, addition of hydroxychloroquine or addition of low-dose prednisolone in the first trimester may be considered. […] These recommendations aim to guide treatment in adults with APS. High-quality evidence is limited, indicating a need for more research.

• #26 Diagnosis and management of antiphospholipid syndrome – Australian Prescriber

  https://australianprescriber.tg.org.au/articles/diagnosis-and-management-of-antiphospholipid-syndrome.html

  If a patient with a history of obstetric manifestations (but no history of thrombotic manifestations) becomes pregnant, both prophylactic-dose low-molecular-weight heparin and low-dose aspirin are recommended for the duration of the pregnancy and postpartum for 6 to 12 weeks. […] If a patient with a history of thrombotic manifestations (but no history of obstetric manifestations) becomes pregnant, therapeutic-dose low-molecular-weight heparin and low-dose aspirin are recommended for the duration of the pregnancy and for 6 to 12 weeks postpartum. […] If catastrophic APS is suspected, the patient should be immediately referred to a hospital for assessment and management. Initial treatment involves anticoagulation and high-dose corticosteroids, and is often followed by plasma exchange and/or rituximab or cyclophosphamide.

• #27 Antiphospholipid Syndrome Treatment & Management: Medical Care, Surgical Care, Consultations

  https://emedicine.medscape.com/article/333221-treatment

  However, studies of these agents in APS patients have largely proved disappointing. […] Rituximab can be considered for recurrent thrombosis despite adequate anticoagulation. A nonrandomized prospective study showed rituximab to be effective for noncriteria aPL manifestations (ie, thrombocytopenia and skin ulcers). […] Guidelines from the American College of Obstetricians and Gynecologists include the following recommendations: Women with APS who have a history of thrombosis in previous pregnancies should receive prophylactic anticoagulation during pregnancy and for 6 weeks postpartum. […] For women with a history of APS and prior thrombosis, anticoagulation with therapeutic-dose low molecular weight heparin (LMWH) and low-dose aspirin is recommended. […] For women with a history of APS without thrombosis but with prior pregnancy loss, prophylactic-dose LMWH and low-dose aspirin are recommended.

• #28 :: JKMS :: Journal of Korean Medical Science

  https://jkms.org/DOIx.php?id=10.3346/jkms.2021.36.e24

  Antiphospholipid syndrome (APS), which is characterized by the presence of antiphospholipid antibodies (aPL), is associated with increased risk of thrombosis and obstetric complications, including preterm delivery and recurrent pregnancy losses. […] Although these patients are usually treated with aspirin and anticoagulants, the optimal treatment in various clinical settings is unclear, as the risk of complications vary among clinical subtypes and the management strategy depends on whether the patient is pregnant or not. […] For asymptomatic aPL positive women, no treatment, low dose aspirin (LDA) or LDA plus anticoagulants can be considered during antepartum and postpartum. […] In obstetric APS patients, preconceptional LDA is recommended. […] LDA plus low molecular weight heparin is administered after confirmation of pregnancy.

• #29 EULAR recommendations for the management of antiphospholipid syndrome in adults | Annals of the Rheumatic Diseases

  https://ard.bmj.com/content/78/10/1296

  For patients with recurrent arterial or venous thrombosis despite adequate treatment, addition of LDA, increase of INR target to 3-4 or switch to low molecular weight heparin may be considered. […] In women with prior obstetric APS, combination treatment with LDA and prophylactic dosage heparin during pregnancy is recommended. […] In patients with recurrent pregnancy complications, increase of heparin to therapeutic dose, addition of hydroxychloroquine or addition of low-dose prednisolone in the first trimester may be considered. […] These recommendations aim to guide treatment in adults with APS. High-quality evidence is limited, indicating a need for more research.

• #30 Antiphospholipid Syndrome Treatment & Management: Medical Care, Surgical Care, Consultations

  https://emedicine.medscape.com/article/333221-treatment

  However, studies of these agents in APS patients have largely proved disappointing. […] Rituximab can be considered for recurrent thrombosis despite adequate anticoagulation. A nonrandomized prospective study showed rituximab to be effective for noncriteria aPL manifestations (ie, thrombocytopenia and skin ulcers). […] Guidelines from the American College of Obstetricians and Gynecologists include the following recommendations: Women with APS who have a history of thrombosis in previous pregnancies should receive prophylactic anticoagulation during pregnancy and for 6 weeks postpartum. […] For women with a history of APS and prior thrombosis, anticoagulation with therapeutic-dose low molecular weight heparin (LMWH) and low-dose aspirin is recommended. […] For women with a history of APS without thrombosis but with prior pregnancy loss, prophylactic-dose LMWH and low-dose aspirin are recommended.

• #31 An update on the management of antiphospholipid syndrome

  https://pmc.ncbi.nlm.nih.gov/articles/PMC7236388/

  Antiphospholipid syndrome (APS) is a systemic autoimmune disorder characterized by recurrent venous or arterial thrombosis with or without pregnancy morbidity in the presence of persistent antiphospholipid (aPL) autoantibodies. Anticoagulation has, until now, formed the cornerstone of treatment but a significant proportion of patients continue to experience thrombosis and pregnancy morbidity despite this treatment. […] In patients with previous thrombosis attributable to APS, anticoagulation has formed the cornerstone of treatment to date. […] Treatment of patients with obstetric APS has improved live birth rates from 40% to 85%, but 15% still fail to achieve a live birth. […] Several nonanticoagulant drugs have demonstrated therapeutic potential in the treatment of the disease, and our increasing understanding of the pathophysiology may lead to a more multifaceted approach to treatment and further reduction of such complications.

• #32 Antiphospholipid Syndrome Treatment & Management: Medical Care, Surgical Care, Consultations

  https://emedicine.medscape.com/article/333221-treatment

  However, studies of these agents in APS patients have largely proved disappointing. […] Rituximab can be considered for recurrent thrombosis despite adequate anticoagulation. A nonrandomized prospective study showed rituximab to be effective for noncriteria aPL manifestations (ie, thrombocytopenia and skin ulcers). […] Guidelines from the American College of Obstetricians and Gynecologists include the following recommendations: Women with APS who have a history of thrombosis in previous pregnancies should receive prophylactic anticoagulation during pregnancy and for 6 weeks postpartum. […] For women with a history of APS and prior thrombosis, anticoagulation with therapeutic-dose low molecular weight heparin (LMWH) and low-dose aspirin is recommended. […] For women with a history of APS without thrombosis but with prior pregnancy loss, prophylactic-dose LMWH and low-dose aspirin are recommended.

• #33 Antiphospholipid Syndrome Treatment & Management: Medical Care, Surgical Care, Consultations

  https://emedicine.medscape.com/article/333221-treatment

  Clopidogrel has anecdotally been reported to be helpful in persons with APS and may be useful in patients allergic to aspirin. […] Perform full anticoagulation with intravenous or subcutaneous heparin followed by warfarin therapy. Based on the most recent evidence, a reasonable target for the international normalized ratio (INR) is 2.0-3.0 for venous thrombosis and 3.0 for arterial thrombosis. Patients with recurrent thrombotic events may require an INR of 3.0-4.0. For severe or refractory cases, a combination of warfarin and aspirin may be used. Treatment for significant thrombotic events in patients with APS is generally lifelong. […] Direct oral anticoagulants (ie, direct thrombin inhibitors and factor Xa inhibitors such as rivaroxaban) have been used in patients with warfarin intolerance/allergy or poor anticoagulant control.

• #34 Antiphospholipid Syndrome and Its Management

  https://www.uspharmacist.com/article/antiphospholipid-syndrome-and-its-management

  DOACs have advantages over warfarin, including fewer drug interactions and the fact that they are prescribed in a fixed dose and tend to have a predictable anticoagulant effect without the need for routine anticoagulation monitoring. […] Although most of the commercially available DOACs compare favorably with warfarin for treatment of VTE and prevention of stroke in patients with atrial fibrillation, there is a lack of published data on the use of DOACs for highly prothrombotic states such as APS, and available data indicate that the DOACs should be avoided in most APS patients, based on the results of several studies. […] The 16th International Congress on Antiphospholipid Antibodies report recommends first-line therapy with a VKA in most patients. […] Most patients with thrombotic APS respond to anticoagulation, but some patients continue to experience clinical events despite anticoagulation. There may be a role for agents such as HCQ, statins, and vitamin D as alternative therapies for APS.

• #35 EULAR recommendations for the management of antiphospholipid syndrome in adults | Annals of the Rheumatic Diseases

  https://ard.bmj.com/content/78/10/1296

  The objective was to develop evidence-based recommendations for the management of antiphospholipid syndrome (APS) in adults. […] Low-dose aspirin (LDA) is recommended for asymptomatic aPL carriers, patients with systemic lupus erythematosus without prior thrombotic or obstetric APS, and non-pregnant women with a history of obstetric APS only, all with high-risk aPL profiles. […] Patients with APS and first unprovoked venous thrombosis should receive long-term treatment with vitamin K antagonists (VKA) with a target international normalised ratio (INR) of 2-3. […] In patients with APS with first arterial thrombosis, treatment with VKA with INR 2-3 or INR 3-4 is recommended, considering the individuals bleeding/thrombosis risk. […] Rivaroxaban should not be used in patients with APS with triple aPL positivity.

• #36 Antiphospholipid Syndrome and Its Management

  https://www.uspharmacist.com/article/antiphospholipid-syndrome-and-its-management

  DOACs have advantages over warfarin, including fewer drug interactions and the fact that they are prescribed in a fixed dose and tend to have a predictable anticoagulant effect without the need for routine anticoagulation monitoring. […] Although most of the commercially available DOACs compare favorably with warfarin for treatment of VTE and prevention of stroke in patients with atrial fibrillation, there is a lack of published data on the use of DOACs for highly prothrombotic states such as APS, and available data indicate that the DOACs should be avoided in most APS patients, based on the results of several studies. […] The 16th International Congress on Antiphospholipid Antibodies report recommends first-line therapy with a VKA in most patients. […] Most patients with thrombotic APS respond to anticoagulation, but some patients continue to experience clinical events despite anticoagulation. There may be a role for agents such as HCQ, statins, and vitamin D as alternative therapies for APS.

• #37 Antiphospholipid syndrome (APS) | Symptoms, treatments

  https://versusarthritis.org/about-arthritis/conditions/antiphospholipid-syndrome/

  To lower your risk of having more blood clots, youre likely to be given warfarin tablets. […] Women with APS and a history of pregnancy complications are usually prescribed low-dose aspirin and daily heparin injections during pregnancy, to reduce the risk of miscarriage. […] Direct-acting oral anti-coagulants, known as DOACs, have been used to treat some people with APS in the past. However, they are now thought to increase your chances of developing blood clots that can interfere with organs such as your lungs and brain. DOACs are not recommended for treating APS, particularly if youve tested positive in all three blood tests.

• #38 Antiphospholipid Syndrome and Its Management

  https://www.uspharmacist.com/article/antiphospholipid-syndrome-and-its-management

  DOACs have advantages over warfarin, including fewer drug interactions and the fact that they are prescribed in a fixed dose and tend to have a predictable anticoagulant effect without the need for routine anticoagulation monitoring. […] Although most of the commercially available DOACs compare favorably with warfarin for treatment of VTE and prevention of stroke in patients with atrial fibrillation, there is a lack of published data on the use of DOACs for highly prothrombotic states such as APS, and available data indicate that the DOACs should be avoided in most APS patients, based on the results of several studies. […] The 16th International Congress on Antiphospholipid Antibodies report recommends first-line therapy with a VKA in most patients. […] Most patients with thrombotic APS respond to anticoagulation, but some patients continue to experience clinical events despite anticoagulation. There may be a role for agents such as HCQ, statins, and vitamin D as alternative therapies for APS.

• #39 Current concepts in the diagnosis and management of antiphospholipid syndrome and ocular manifestations | Journal of Ophthalmic Inflammation and Infection | Full Text

  https://joii-journal.springeropen.com/articles/10.1186/s12348-021-00240-8

  Hydroxychloroquine (HCQ) is an antimalarial drug that is typically used in SLE patients which has anti-inflammatory as well as anti-thrombotic effects. It has been shown that HCQ has anti-thrombotic effects based on inhibition of glycoprotein IIb/IIIa (GPIIb/IIIa) expression on aPL activated platelets, preventing aPL-2GPI-phospholipid formation, and aPL-mediated Annexin A5 shield disruption. Furthermore, HCQ is known to have immunomodulatory effects including prevention of Toll-like receptor 3 (TLR3), Toll-like receptor 7 (TLR7) and Toll-like receptor 9 (TLR9) activation as well as reduction in IFN signature and aPL titers. On the basis of anti-thrombotic and immunomodulatory properties, HCQ can be considered as an adjunctive therapy for APS patients who have recurrent thrombotic events despite being on adequate anticoagulation therapy. However, further clinical studies are needed supporting the clinical antithrombotic effects of HCQ in patients with APS.

• #40 Antiphospholipid Syndrome and Potential New Treatments | HSS

  https://www.hss.edu/conditions_top-ten-series-antiphospholipid-syndrome-potential-new-treatments.asp

  Recent studies based on newly understood mechanisms suggest new treatments that target different areas of coagulation system (that is, medications that interfere with clotting factors) or inflammation pathway (that is, medications that interact with ones immune system with the goal of decreasing or eliminating the production and effects of the aPL). […] Hydroxychloroquine (Plaquenil) may be used as an adjunctive therapy (supportive treatment used together with the primary treatment) in APS patients with difficult-to-control aPL-related clinical problems. […] Currently, there is no definitive evidence that statins prevent clots in APS patients but they can be considered in difficult-to-treat APS patients. […] Preliminary small scale studies show that rituximab may have a role in difficult-to treat APS patients, possibly in those with hematologic and small vessel involvement.

• #41 Antiphospholipid Syndrome and Its Management

  https://www.uspharmacist.com/article/antiphospholipid-syndrome-and-its-management

  The 16th International Congress on Antiphospholipid Antibodies report states that statins may be beneficial in the primary and secondary prevention of arterial thrombosis in patients with aPLs/APS but cannot be recommended in patients in the absence of hyperlipidemia. […] The 16th International Congress on Antiphospholipid Antibodies report recommends correcting vitamin D deficiency and insufficiency in all patients based on guidelines for the general population. […] VKAs remain the standard of care for APS management in most cases; DOAC use is not generally recommended, owing to limited evidence. Aspirin use is controversial and depends on individual risk. More research is needed to establish therapeutic recommendations for APS treatment.

• #42 Antiphospholipid Syndrome Treatment & Management: Medical Care, Surgical Care, Consultations

  https://emedicine.medscape.com/article/333221-treatment

  However, studies of these agents in APS patients have largely proved disappointing. […] Rituximab can be considered for recurrent thrombosis despite adequate anticoagulation. A nonrandomized prospective study showed rituximab to be effective for noncriteria aPL manifestations (ie, thrombocytopenia and skin ulcers). […] Guidelines from the American College of Obstetricians and Gynecologists include the following recommendations: Women with APS who have a history of thrombosis in previous pregnancies should receive prophylactic anticoagulation during pregnancy and for 6 weeks postpartum. […] For women with a history of APS and prior thrombosis, anticoagulation with therapeutic-dose low molecular weight heparin (LMWH) and low-dose aspirin is recommended. […] For women with a history of APS without thrombosis but with prior pregnancy loss, prophylactic-dose LMWH and low-dose aspirin are recommended.

• #43 Current concepts in the diagnosis and management of antiphospholipid syndrome and ocular manifestations | Journal of Ophthalmic Inflammation and Infection | Full Text

  https://joii-journal.springeropen.com/articles/10.1186/s12348-021-00240-8

  Statins, also known as hydroxymethylglutaryl coenzyme A (HMG-CoA) reductase inhibitors, are a type of cholesterol lowering agent. In addition to a lipid-lowering effect, statins have demonstrated anti-thrombotic and anti-inflammatory properties such as inhibition of tissue factor production in endothelial cells and prevention of anti-2GPI antibody-mediated endothelial adherence. Fluvastatin treatment has been proposed to decrease proinflammatory and prothrombotic mediators in aPL positive patients and to inhibit several mediators in monocytes that may be pro-thrombotic in APS patients. […] Rituximab is a chimeric monoclonal antibody against CD 20 expressed on B cells that was originally developed for the treatment of non-Hodgkins B-cell lymphoma. In the last several years, B-cell targeting therapies have been approved for the treatment of various autoimmune diseases. Anti-CD20 monoclonal therapy has been shown to decrease aPL titers and prevent new thrombotic events. Moreover, in a non-randomized prospective pilot study, rituximab was found to be effective in the treatment of non-criteria aPL manifestations in persistently aPL positive patients. It has also been a safe and effective therapeutic option for patients with refractory catastrophic APS.

• #44 An update on the biologics for the treatment of antiphospholipid syndrome

  https://pmc.ncbi.nlm.nih.gov/articles/PMC10237350/

  This review focuses on biologics as potential targeted therapies for APS based on its underlying mechanisms. We will discuss possible biologics beyond antithrombotic agents. […] Rituximab is recommended for use in APS, particularly in cases of refractory CAPS, or CAPS with noncriteria manifestations such as acute kidney injury and severe thrombocytopenia. […] Eculizumab can improve outcomes of refractory CAPS described in previous case reports. When rituximab and immunoglobulins fail to improve platelet count and renal function, eculizumab may be an effective alternative supplement. […] Belimumab may be beneficial in managing APS patients with high thrombotic risk or aPL-positive patients with microthrombotic manifestations. […] Daratumumab may be suitable for APS patients who are unresponsive to anticoagulant therapy and standard immunosuppression. […] Anti-TNF- blockers were all well tolerated without adverse effects.

• #45 Current concepts in the diagnosis and management of antiphospholipid syndrome and ocular manifestations | Journal of Ophthalmic Inflammation and Infection | Full Text

  https://joii-journal.springeropen.com/articles/10.1186/s12348-021-00240-8

  Eculizumab is a humanized monoclonal antibody against complement C5 that inhibits C5 cleavage into C5a and C5b, thus preventing membrane attack complex formation. It is currently used for paroxysmal nocturnal hemoglobinuria. C5 is a strong proinflammatory, chemotactic, and anaphylatoxic molecule that also shows prothrombotic effect by regulating many mediators from numerous cells. It induces tissue factor expression on endothelial cells, neutrophils, and monocytes. Eculizumab has been used in treatment of refractory CAPS and aPL-mediated acute thrombotic microangiopathy after renal transplantations. […] Inhibition of mammalian target of rapamycin (mTOR) pathway can prevent APS-related vasculopathy by inhibiting proliferation of endothelial and vascular smooth muscle cells. Sirolimus, an mTOR inhibitor, reduced the development of intimal hyperplasia and showed better graft survival in patients with APS who underwent renal transplantation. It has also been shown that anti-2GPI results in mTOR activation as well as tissue factor and Interleukin-8 (IL-8) expression in monocytes. Based on these findings, mTOR inhibition can be considered as a useful therapeutic option in the future in terms of preventing aPL-mediated thrombosis and inflammation in APS patients.

• #46 An update on the biologics for the treatment of antiphospholipid syndrome

  https://pmc.ncbi.nlm.nih.gov/articles/PMC10237350/

  This review focuses on biologics as potential targeted therapies for APS based on its underlying mechanisms. We will discuss possible biologics beyond antithrombotic agents. […] Rituximab is recommended for use in APS, particularly in cases of refractory CAPS, or CAPS with noncriteria manifestations such as acute kidney injury and severe thrombocytopenia. […] Eculizumab can improve outcomes of refractory CAPS described in previous case reports. When rituximab and immunoglobulins fail to improve platelet count and renal function, eculizumab may be an effective alternative supplement. […] Belimumab may be beneficial in managing APS patients with high thrombotic risk or aPL-positive patients with microthrombotic manifestations. […] Daratumumab may be suitable for APS patients who are unresponsive to anticoagulant therapy and standard immunosuppression. […] Anti-TNF- blockers were all well tolerated without adverse effects.

• #47 Current concepts in the diagnosis and management of antiphospholipid syndrome and ocular manifestations | Journal of Ophthalmic Inflammation and Infection | Full Text

  https://joii-journal.springeropen.com/articles/10.1186/s12348-021-00240-8

  Eculizumab is a humanized monoclonal antibody against complement C5 that inhibits C5 cleavage into C5a and C5b, thus preventing membrane attack complex formation. It is currently used for paroxysmal nocturnal hemoglobinuria. C5 is a strong proinflammatory, chemotactic, and anaphylatoxic molecule that also shows prothrombotic effect by regulating many mediators from numerous cells. It induces tissue factor expression on endothelial cells, neutrophils, and monocytes. Eculizumab has been used in treatment of refractory CAPS and aPL-mediated acute thrombotic microangiopathy after renal transplantations. […] Inhibition of mammalian target of rapamycin (mTOR) pathway can prevent APS-related vasculopathy by inhibiting proliferation of endothelial and vascular smooth muscle cells. Sirolimus, an mTOR inhibitor, reduced the development of intimal hyperplasia and showed better graft survival in patients with APS who underwent renal transplantation. It has also been shown that anti-2GPI results in mTOR activation as well as tissue factor and Interleukin-8 (IL-8) expression in monocytes. Based on these findings, mTOR inhibition can be considered as a useful therapeutic option in the future in terms of preventing aPL-mediated thrombosis and inflammation in APS patients.

• #48 Natural supplements to support Antiphospholipid Syndrome

  https://www.autoimmunutrition.com/post/natural-supplements-to-support-antiphospholipid-syndrome

  The standard medical care for APS may involve antiplatelet or anticoagulation measures such as vitamin K antagonists e.g. warfarin and supporting the cardiovascular system. […] Since it is very important in APS to promote antithrombotic, anti-inflammatory and antioxidant pathways, the evidence behind the uses and benefits of natural supplements for APS should be considered: […] The 16th International Congress on Antiphospholipid Antibodies Task Force Report in 2020, recommended that vitamin D deficiency be corrected in all APS patients. […] In a 2018 clinical trial, 16 weeks of omega-3 supplementation improved endothelial function in patients with primary APS. […] 36 patients with APS who received CoQ10 supplementation in APS showed improved endothelial function and mitochondrial performance and decreased prothrombotic and proinflammatory mediators.

• #49 Natural supplements to support Antiphospholipid Syndrome

  https://www.autoimmunutrition.com/post/natural-supplements-to-support-antiphospholipid-syndrome

  Studies, mostly in-vivo, conclude that ginger possesses an anti-thrombotic property, probably through inhibition of platelet function. […] One study evaluated the effect of quercetin in a cohort of patients with persistently elevated antiphospholipid antibodies. Oral administration of quercetin decreased platelet-dependent thrombin generation by 64%. […] Randomised clinical trials have demonstrated that supplementation with Vitamin E reduced oxidative stress markers and thrombin generation in patients with APS. […] EGCG significantly blocked the effects of the anti-2 glycoprotein complex concluding anti-thrombosis and anti-inflammatory properties. […] Curcumin has been found to dampen platelet activation and reduce platelet aggregation to endothelial cells. […] Resveratrol improved endothelial function, in 19 patients with obesity and has been proposed to help with stroke as it improves blood flow, blood pressure, body mass index and lipid profile.

• #50 Natural supplements to support Antiphospholipid Syndrome

  https://www.autoimmunutrition.com/post/natural-supplements-to-support-antiphospholipid-syndrome

  Unfortunately, there is still scarce human clinical data on the benefits, safety and dosage levels of natural supplements for APS. Natural supplements are not substitutes for medical care in APS. They may however be very beneficial in supporting the patient’s underlying biology through anti-inflammatory and antioxidant mechanisms. […] Patients should always consult with their medical professionals and work with a knowledgeable healthcare practitioner who can advise on supplement-medicine interactions and the correct dosages to use. This is particularly important for blood thinning medications in APS, due to their many interactions with herbal supplements. […] It is important to note that Vitamin K supplements should not be taken by patients with APS due to the increased risk of clotting.

• #51 Antiphospholipid Syndrome: Practice Essentials, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/333221-overview

  In general, treatment regimens for APS must be individualized according to the patient’s current clinical status and history of thrombotic events. […] For patients with APS, prophylaxis is needed during surgery or hospitalization, as is management of any associated autoimmune disease. Low-dose aspirin is used widely in this setting; although its effectiveness remains unproven. For thrombosis, perform full anticoagulation with intravenous or subcutaneous heparin followed by warfarin therapy. […] Catastrophic APS (CAPS) is a rare, serious, and often fatal manifestation characterized by multiorgan infarctions over a period of days to weeks. Mortality rates of 50% have been reported; however, with triple therapy (anticoagulation, corticosteroids, plasma exchange and/or intravenous immunoglobulin) data from an international registry showed a mortality rate of 28.6%. […] Direct oral anticoagulants in antiphospholipid syndrome with venous thromboembolism: Impact of the European Medicines Agency guidance. […] Rivaroxaban vs warfarin in high-risk patients with antiphospholipid syndrome.

• #52 Update on the Diagnosis and Treatment of the Antiphospholipid Syndrome

  https://www.emjreviews.com/rheumatology/article/update-on-the-diagnosis-and-anticoagulant-treatment-of-the-antiphospholipid-syndrome/

  Hydroxychloroquine has anti-inflammatory and anti-thrombotic properties and is recommended by recent consensus guidelines as primary thromboprophylaxis in patients with aPL, SLE, and no contraindications. […] Statins have pleiotropic effects, including anti-inflammatory and anti-thrombotic properties. […] Rituximab is a monoclonal antibody against CD20 located mainly on B-lymphocytes. […] Treatment of CAPS should aim at the control of any precipitating factor, as well as the prevention and treatment of thrombosis. […] In a proposed treatment algorithm for CAPS, prompt use of anticoagulation (usually intravenous UFH) and high dose corticosteroids represented the first-line option.

• #53 Antiphospholipid Syndrome Treatment & Management: Medical Care, Surgical Care, Consultations

  https://emedicine.medscape.com/article/333221-treatment

  Warfarin is contraindicated in pregnancy. Breastfeeding women may use heparin and warfarin. […] Unfortunately, current treatment fails to prevent complications in 20-30% of APS pregnancies. […] In patients with a history of SLE and APS, hydroxychloroquine should be used during pregnancy. […] Patients with catastrophic antiphospholipid syndrome (CAPS) are generally very ill, often with active SLE. […] Improved mortality has been reported with triple therapy consisting of anticoagulation, corticosteroids, and plasma exchange and/or intravenous immunoglobulin. […] Placement of an inferior vena cava (IVC) filter may be considered in patients with APS who require cessation of anticoagulation or who continue to experience thrombotic complications despite maximal anticoagulation.

• #54 Lupus anticoagulants and antiphospholipid antibodies Information | Mount Sinai – New York

  https://www.mountsinai.org/health-library/diseases-conditions/lupus-anticoagulants-and-antiphospholipid-antibodies

  If you have SLE and APS, your provider may also recommend that you take hydroxychloroquine. […] Currently, other types of blood thinning medicines are not recommended. […] Treatment for CAPS that involves a combination of anticoagulation therapy, high doses of corticosteroids, and plasma exchange has been effective in most people. Sometimes IVIG, rituximab or eculizumab is also used for severe cases. […] You will be prescribed blood-thinning medicines (such as heparin and warfarin) to help prevent blood clots: After surgery, After a bone fracture, With active cancer, When you need to sit or lie down for long periods of time, such as during a hospital stay or recovering at home. […] You may also need to take blood thinners for 3 to 4 weeks after surgery to lower your risk for blood clots.

• #55 Patient education: Antiphospholipid syndrome (Beyond the Basics) – UpToDate

  https://www.uptodate.com/contents/antiphospholipid-syndrome-beyond-the-basics

  Monitoring of warfarin — Careful monitoring and periodic adjustments in the warfarin dose are typically needed to maintain a level that prevents clots but that does not cause significant bleeding. The blood test used to measure the effects of warfarin is called the prothrombin time (called pro time, or PT). The PT is a laboratory test that measures the time it takes for the clotting mechanism to progress. It is particularly sensitive to the clotting factors affected by warfarin. The PT is used to compute a value known as the International Normalized Ratio (INR). The INR is a way of expressing the PT in a standardized way; this ensures that results obtained by different laboratories can be reliably compared. […] The longer it takes the blood to clot, the higher the PT and INR. The target INR range depends upon the clinical situation. In most cases, the target range is 2 to 3. If the INR is below the target range (ie, under-anticoagulated), there is a risk of clotting. If, on the other hand, the INR is above the target range (ie, over-anticoagulated), there is an increased risk of bleeding.

• #56 Patient education: Antiphospholipid syndrome (Beyond the Basics) – UpToDate

  https://www.uptodate.com/contents/antiphospholipid-syndrome-beyond-the-basics

  Monitoring of warfarin — Careful monitoring and periodic adjustments in the warfarin dose are typically needed to maintain a level that prevents clots but that does not cause significant bleeding. The blood test used to measure the effects of warfarin is called the prothrombin time (called pro time, or PT). The PT is a laboratory test that measures the time it takes for the clotting mechanism to progress. It is particularly sensitive to the clotting factors affected by warfarin. The PT is used to compute a value known as the International Normalized Ratio (INR). The INR is a way of expressing the PT in a standardized way; this ensures that results obtained by different laboratories can be reliably compared. […] The longer it takes the blood to clot, the higher the PT and INR. The target INR range depends upon the clinical situation. In most cases, the target range is 2 to 3. If the INR is below the target range (ie, under-anticoagulated), there is a risk of clotting. If, on the other hand, the INR is above the target range (ie, over-anticoagulated), there is an increased risk of bleeding.

• #57 Patient education: Antiphospholipid syndrome (Beyond the Basics) – UpToDate

  https://www.uptodate.com/contents/antiphospholipid-syndrome-beyond-the-basics

  Antiplatelet treatments — Aspirin is another medication sometimes recommended for people with APS. Aspirin inhibits the clumping of platelets; platelets are tiny cell fragments in the blood that have a role in blood clotting. Under normal circumstances, platelets clump together and help form blood clots to stop bleeding when needed. […] Aspirin is sometimes used in addition to warfarin. It is not usually used as a replacement for warfarin, especially in people with a history of serious and recurrent blood clots. […] Treatment before and during surgery — Anticoagulant and antiplatelet treatments may need to be adjusted before, during, and after surgery or other procedures that have a risk of bleeding. The goal of treatment adjustments is to minimize the risk of new blood clots as well as the risk of excessive bleeding. This usually involves stopping warfarin several days before surgery.

• #58 Patient education: Antiphospholipid syndrome (Beyond the Basics) – UpToDate

  https://www.uptodate.com/contents/antiphospholipid-syndrome-beyond-the-basics

  The person may be given heparin before and after surgery, depending upon several factors (the person’s previous history of blood clots, type of surgery, etc). Warfarin can usually be restarted within 12 hours after surgery. A person should speak with the provider who prescribes their warfarin to determine the best treatment regimen (and the timing) before and after surgery. […] TREATMENT OF LOW PLATELETS […] People with mild thrombocytopenia (low platelet count) due to antiphospholipid syndrome (APS) usually do not require treatment. In more severe cases, medications (including steroids or immune globulins) are used to help increase the platelet count. […] ANTIPHOSPHOLIPID SYNDROME AND PREGNANCY […] Several treatments are available to reduce these risks. Treatment depends upon the person’s history of blood clots, miscarriage, stillbirth, and preeclampsia, as well as current antibody levels. For people who have antiphospholipid antibodies (aPL) but no history of blood clots or miscarriage, treatment may or may not be recommended.

• #59 Patient education: Antiphospholipid syndrome (Beyond the Basics) – UpToDate

  https://www.uptodate.com/contents/antiphospholipid-syndrome-beyond-the-basics

  The person may be given heparin before and after surgery, depending upon several factors (the person’s previous history of blood clots, type of surgery, etc). Warfarin can usually be restarted within 12 hours after surgery. A person should speak with the provider who prescribes their warfarin to determine the best treatment regimen (and the timing) before and after surgery. […] TREATMENT OF LOW PLATELETS […] People with mild thrombocytopenia (low platelet count) due to antiphospholipid syndrome (APS) usually do not require treatment. In more severe cases, medications (including steroids or immune globulins) are used to help increase the platelet count. […] ANTIPHOSPHOLIPID SYNDROME AND PREGNANCY […] Several treatments are available to reduce these risks. Treatment depends upon the person’s history of blood clots, miscarriage, stillbirth, and preeclampsia, as well as current antibody levels. For people who have antiphospholipid antibodies (aPL) but no history of blood clots or miscarriage, treatment may or may not be recommended.

• #60 Antiphospholipid Syndrome: Practice Essentials, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/333221-overview

  In general, treatment regimens for APS must be individualized according to the patient’s current clinical status and history of thrombotic events. […] For patients with APS, prophylaxis is needed during surgery or hospitalization, as is management of any associated autoimmune disease. Low-dose aspirin is used widely in this setting; although its effectiveness remains unproven. For thrombosis, perform full anticoagulation with intravenous or subcutaneous heparin followed by warfarin therapy. […] Catastrophic APS (CAPS) is a rare, serious, and often fatal manifestation characterized by multiorgan infarctions over a period of days to weeks. Mortality rates of 50% have been reported; however, with triple therapy (anticoagulation, corticosteroids, plasma exchange and/or intravenous immunoglobulin) data from an international registry showed a mortality rate of 28.6%. […] Direct oral anticoagulants in antiphospholipid syndrome with venous thromboembolism: Impact of the European Medicines Agency guidance. […] Rivaroxaban vs warfarin in high-risk patients with antiphospholipid syndrome.

• #61

  https://www.nhs.uk/conditions/antiphospholipid-syndrome/

  Although there’s no cure for APS, the risk of developing blood clots can be greatly reduced if it’s correctly diagnosed. […] An anticoagulant medicine, such as warfarin, or an antiplatelet, such as low-dose aspirin, is usually prescribed. […] These reduce the likelihood of unnecessary blood clots forming, but still allow clots to form when you cut yourself. […] Treatment with these medications can also improve a pregnant woman’s chance of having a successful pregnancy. […] With treatment, it’s estimated there’s about an 80% chance of having a successful pregnancy. […] Most people respond well to treatment and can lead normal, healthy lives. […] But a small number of people with APS continue to experience blood clots despite extensive treatment.

• #62 An update on the management of antiphospholipid syndrome

  https://pmc.ncbi.nlm.nih.gov/articles/PMC7236388/

  Antiphospholipid syndrome (APS) is a systemic autoimmune disorder characterized by recurrent venous or arterial thrombosis with or without pregnancy morbidity in the presence of persistent antiphospholipid (aPL) autoantibodies. Anticoagulation has, until now, formed the cornerstone of treatment but a significant proportion of patients continue to experience thrombosis and pregnancy morbidity despite this treatment. […] In patients with previous thrombosis attributable to APS, anticoagulation has formed the cornerstone of treatment to date. […] Treatment of patients with obstetric APS has improved live birth rates from 40% to 85%, but 15% still fail to achieve a live birth. […] Several nonanticoagulant drugs have demonstrated therapeutic potential in the treatment of the disease, and our increasing understanding of the pathophysiology may lead to a more multifaceted approach to treatment and further reduction of such complications.

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