Antiphospholipid Syndrome Handbook

Other symptoms of venous or arterial thrombosis may also develop. Patients with autoantibodies to phospholipid-bound prothrombin may have levels of circulating prothrombin that are low enough to increase risk of bleeding. Some patients have thrombocytopenia. In a small proportion of patients with APS, widespread thromboses occur in small vessels supplying multiple organs, often causing neurologic defects.

Its treatment includes high-dose corticosteroids, anticoagulation, plasmapheresis, and sometimes rituximab or eculizumab. PTT testing is done in patients who are expected to undergo an invasive procedure or in those with unexplained bleeding or clotting. The lupus anticoagulant is suspected if the PTT is prolonged and does not correct immediately upon 1: Heparin , warfarin except in pregnant women , and aspirin have been used for prophylaxis and treatment.

It is possible, but not yet certain, that the direct oral anticoagulant DOAC inhibitors of either thrombin dabigatran or factor Xa eg, rivaroxaban , apixaban can be used in place of heparin or warfarin for this disorder. Tests used to detect cancer include imaging tests, serum tumor markers, and biopsy; one or more may be indicated in patients with a suggestive history or physical or laboratory findings. Which of the following is the serum tumor marker most likely used to detect colon cancer? Tap to switch to the Consumer Version.

This is the Professional Version. Click here for the Consumer Version. Several LMWH products are now available for clinical use. Dosing requirements are individualized for each product The advantages of LMWH over unfractionated heparin are reviewed separately. Unfractionated heparin is preferred to LMWH in certain circumstances. Unfractionated heparin can be reversed quickly with protamine while LMWH is not completely reversible with this approach. The major condition in which hemorrhage is due to APS is when antibodies to prothrombin are present.

Following stabilization of the patient, warfarin is begun. Warfarin is the standard of care for the chronic management of patients with APS who are not pregnant. INR should be maintained between 2. A monotonous diet with only slight variations in the amount of vitamin K intake, intensification of monitoring when a different medication has to be used, and above all, patient education on the importance of close monitoring are crucial for the APS management to succeed. Aspirin is of minimal or no benefit for the prevention of thrombotic APS manifestations in patients who have experienced previous events according to retrospective series In addition to its antiplatelet effects, low dose aspirin ASA 50 to mg enhances leukocyte-derived interleukin-3 production, which stimulates normal trophoblast growth and hormone expression Retrospective and prospective observational studies and controlled trials of aspirin for the prevention of thrombotic events in people with aPL with no history of arterial or venous thromboembolism have had disparate results 82 , Asymptomatic individuals who are persistently positive for aPL have a low annual incidence of acute thrombosis.

These individuals do not benefit from low-dose aspirin. Thrombotic events in this population are unlikely in the absence of additional risk factors for thrombosis. It has anecdotally been reported to be helpful in patients with APS and may be useful in those allergic to aspirin. Its use is not advised for the treatment of APS Treatments in APS are directed at modulating the final event or second hit. Treatments that modulate the early effects of aPL on target cells — that is monocytes or endothelial cells first hit — would be more beneficial and potentially less harmful than current treatments.

The current antithrombotic approach to aPL-positive patients may be replaced by an immunomodulatory approach in the future as our understanding of the mechanisms of aPL-mediated thrombosis improves.

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Understanding the molecular mechanisms triggered by aPL and identifying biomarkers released as a consequence of cell activation may help us design new ways to treat clinical manifestations in APS. The main target recognized by aPL binds to endothelial cells and monocytes through its fifth domain. Recently, Ioannou et al. Nevertheless, human studies are needed to establish the safety and efficacy of such a treatment 89 , It provides fast and continuous platelet inhibition since pre-stimulation of platelets by agonists leads to the exposure of phosphatidylserine on the outer membrane of the cell.

HCQ could be used in patients with APS and thrombosis as a second-line agent together with anticoagulation therapy. We still do not have a study result for a consistent recommendation for HCQ in APS although, in SLE, it is known to reduce the thrombotic risk, including during pregnancy. RTX has been shown to be a good treatment for life-threatening CAPS in a few patients and case reports suggest it may be successful in patients with aPL, autoimmune-mediated thrombocytopenia, and hemolytic anemia. Recently, an uncontrolled and nonrandomized pilot study suggested that the safety of rituximab in aPL-positive patients with non-criteria manifestations of APS is consistent with the safety profile of rituximab.

Despite causing no substantial change in aPL profiles, rituximab may be effective in controlling some but not all non-criteria manifestations of APS Prophylaxis is recommended for prenatal and postpartum women with APS with no history of thrombosis and full anticoagulation for those with a history of thrombosis. The administration of low-dose ASA alone for the prevention of fetal loss in women with APS has been associated with an increased frequency of successful pregnancy outcome in some studies but was no better than supportive care in others Low-dose ASA can be stopped any time after 36 weeks of gestation and, ideally, should be stopped 7 to 10 days before delivery as some studies have reported a slight increase in mostly minor perioperative bleeding with continuation of the drug In women with a past history of serious arterial thrombotic complications such as stroke or myocardial infarction, the potential benefit of continuing ASA through labor and delivery outweighs the small risk of incisional bleeding.

Aspirin is not usually stopped in these patients. Use of low-dose ASA has not been associated with either premature closure of the ductusarteriosusor or an increase in significant postpartum events. Patients with pregnancy loss receive prophylactic subcutaneous heparin preferably LMWH and low-dose aspirin. Therapy is withheld at the time of delivery, restarted after delivery, and continued for at least for 6—12 weeks postpartum.

Warfarin Coumadin is contraindicated in pregnancy, mainly between 6 and 9 weeks. In a few centers, it is used between 15 and 35 weeks, before and after LMWH. Patients with a history of thrombosis receive therapeutic doses of heparin during pregnancy combined with low-dose aspirin. Long-term anticoagulation is then continued postpartum. Corticosteroids have not been shown to be effective for patients with primary APS and have been shown to increase maternal morbidity and fetal prematurity rates.

Breastfeeding women may use heparin, low-dose aspirin, and warfarin. Turn recording back on.

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National Center for Biotechnology Information , U. Table 1 Antiphospholipid antibody aPL -mediated pathogenic mechanisms. Clinical manifestations Thromboses are one of the hallmarks of APS, and venous thrombosis, or embolism, is the most frequent manifestation 2. Classification and diagnosis The current guidelines for the classification of APS rely upon clinical and laboratory criteria. Table 2 Revised classification criteria for the antiphospholipid syndrome. Catastrophic antiphospholipid syndrome CAPS was first described by Asherson in 58 and is characterized by multiple vascular occlusive events presenting over a short period of time in aPL-positive patients.

Treatment APS is characterized by recurrent thrombotic events that have not been properly managed. Prophylactic therapy Eliminate other risk factors such as oral contraceptives, smoking, hypertension, hyperhomocysteinemia, or hyperlipidemia. Current treatment of thrombosis Treatments in APS are directed at modulating the final event or second hit. Rituximab RTX RTX has been shown to be a good treatment for life-threatening CAPS in a few patients and case reports suggest it may be successful in patients with aPL, autoimmune-mediated thrombocytopenia, and hemolytic anemia.

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Obstetric considerations Prophylaxis is recommended for prenatal and postpartum women with APS with no history of thrombosis and full anticoagulation for those with a history of thrombosis. Caps These patients are generally very ill, often with active SLE. Treatment with intensive anticoagulation, plasma exchange, and corticosteroids appears beneficial, but no controlled trials have been done. Intravenous immunoglobulin may be of some benefit and cyclophosphamide may be considered in selected cases, especially in SLE-associated CAPS.

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Antiphospholipid Syndrome Handbook - AJNR Blog

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New autoantigens in the antiphospholipid syndrome.

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