Thrombotic antiphospholipid syndrome: A practical guide to diagnosis and management

Sayar, Zara; Moll, Rachel; Isenberg, David; Cohen, Hannah

doi:10.1016/j.thromres.2020.10.010

Cited by 55 publications

(59 citation statements)

References 96 publications

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“…The guidance from important groups in the field, such as the International Congress on Antiphospholipid Antibodies and the International Society on Thrombosis and Haemostasis, suggests that DOACs can be considered for the treatment of an initial venous thrombosis in a patient who is single- or double-positive for aPL. 11 However, these groups recommend against DOACs for patients with triple-positive aPL, arterial thrombosis, or recurrent venous thromboses. A recent meta-analysis of four important RCTs that compared DOACs with vitamin K antagonists (VKAs) was recently published.…”

Section: Discussionmentioning

confidence: 99%

An arm and a leg: A case of rheumatoid vasculitis and antiphospholipid antibody syndrome

Ghadiali

Talwar

Ligon

2021

SAGE Open Medical Case Reports

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Rheumatoid vasculitis is a rare extra-articular complication of rheumatoid arthritis. The most common manifestation is cutaneous; however, it can manifest in various organ systems and is associated with a high degree of morbidity and mortality. Diagnosis is challenging, and there are no validated diagnostic or classification criteria. Most cases should be confirmed with tissue biopsy when possible given the severity of disease and the extent of immunosuppression required to treat this condition. We report the case of a 54-year-old white woman with long-standing, uncontrolled, and seropositive rheumatoid arthritis with a history of elevated anticardiolipin IgG and IgM antibodies who presented with acute stenosis of her left femoral artery which ultimately required a left above-the-knee amputation. Histopathology revealed findings consistent with vasculitis and thrombosis, and subsequent imaging revealed multifocal arterial and venous thromboses. She was diagnosed with rheumatoid vasculitis and antiphospholipid antibody syndrome, and was treated with high-dose glucocorticoids, cyclophosphamide, and warfarin. Rheumatoid vasculitis is a rare but devastating complication of rheumatoid arthritis, and vigilance for this condition must be maintained, especially in patients with long-standing, seropositive disease.

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Section: Discussionmentioning

confidence: 99%

An arm and a leg: A case of rheumatoid vasculitis and antiphospholipid antibody syndrome

Ghadiali

Talwar

Ligon

2021

SAGE Open Medical Case Reports

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“…Movement disorders Laboratory findings Brain MRI findings Treatment often combined, movement disorders have been described in APS without SLE, and in rare cases of SLE without antiphospholipid antibodies (aPL) (2,3). APS is usually defined by the occurrence of recurrent thrombosis and/or pregnancy complications in the presence of persistent aPL (lupus anticoagulant, anti-cardiolipin or anti-β2-glycoprotein-1, present on two occasions, at least 12 weeks apart) (1,4). Movement disorders may be a consequence of ischemic strokes, but often occur without any evidence of ischemic damage.…”

Section: Causementioning

confidence: 99%

Treatable Hyperkinetic Movement Disorders Not to Be Missed

et al. 2021

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Hyperkinetic movement disorders are characterized by the presence of abnormal involuntary movements, comprising most notably dystonia, chorea, myoclonus, and tremor. Possible causes are numerous, including autoimmune disorders, infections of the central nervous system, metabolic disturbances, genetic diseases, drug-related causes and functional disorders, making the diagnostic process difficult for clinicians. Some diagnoses may be delayed without serious consequences, but diagnosis delays may prove detrimental in treatable disorders, ranging from functional disabilities, as in dopa-responsive dystonia, to death, as in Whipple's disease. In this review, we focus on treatable disorders that may present with prominent hyperkinetic movement disorders.

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“…Although the use of DOACs represents a potential advance for the treatment and prophylaxis of thrombus in children and adolescents [126][127][128][129][130][131], guidance from professional organizations recommends strictly avoiding the use of DOACs in patients with APS with arterial, small vessel, and recurrent thrombosis or cardiac valvular disease and in those with triple aPL positivity [88,106,112]. This recommendation was based on clinical trials with negative outcomes for DOAC-treated patients.…”

Section: Direct Oral Anticoagulantsmentioning

confidence: 99%

Managing Antiphospholipid Syndrome in Children and Adolescents: Current and Future Prospects

et al. 2021

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Pediatric antiphospholipid syndrome (APS) is a rare acquired multisystem autoimmune thromboinflammatory condition characterized by thrombotic and non-thrombotic clinical manifestations. APS in children and adolescents typically presents with large-vessel thrombosis, thrombotic microangiopathy, and, rarely, obstetric morbidity. Non-thrombotic clinical manifestations are frequently seen in pediatric APS and may be present even before the vascular thrombotic events occur. We review insights into the pathogenesis of APS and discuss potential targets for therapy. The identification of multiple immunologic abnormalities in patients with APS reveals molecular targets for current or future treatment. Management strategies, especially for APS in adolescents, require screening for additional prothrombotic risk factors and consideration of counseling regarding contraceptive strategies, lifestyle recommendations, treatment adherence, and mental health issues associated with this autoimmune thrombophilia. The main goal of therapy in pediatric APS is the prevention of thrombosis. The management of acute thrombosis events in children and adolescents is the same as for primary APS, which involves isolated occurrences, and secondary APS, which is seen in association with another autoimmune disease, e.g., systemic lupus erythematosus. A pediatric hematologist should be consulted so other differential thrombophilic conditions can be eliminated. Therapy includes unfractionated heparin or low-molecular-weight heparin followed by vitamin K antagonists. Treatment of catastrophic APS involves triple therapy (anticoagulation, intravenous corticosteroid pulse therapy, and plasma exchange) and may include intravenous immunoglobulin for children and adolescents with this condition. New drugs such as eculizumab and sirolimus seem to be promising drugs for APS.

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Thrombotic antiphospholipid syndrome: A practical guide to diagnosis and management

Cited by 55 publications

References 96 publications

An arm and a leg: A case of rheumatoid vasculitis and antiphospholipid antibody syndrome

An arm and a leg: A case of rheumatoid vasculitis and antiphospholipid antibody syndrome

Treatable Hyperkinetic Movement Disorders Not to Be Missed

Managing Antiphospholipid Syndrome in Children and Adolescents: Current and Future Prospects

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