Antiphospholipid syndrome (APS) Anna Rudaja MFIV 3.gr.
Characteristicclinically : recurrent venousor arterial thrombosis and/orfetal losslaboratory : persistentlyelevated levels of antibodiesdirected against membraneanionic phospholipids (ie,anticardiolipin [aCL]antibody,antiphosphatidylserine) ortheir associated plasmaproteins, predominantly beta-2glycoprotein I (apolipoproteinH); or evidence of a circulatinganticoagulant
Pathophysiology1. defect in cellular 1. oxidized beta-2 glycoprotein I is able to apoptosis bind to and activate2. membrane dendritic cells in a manner similar to activation phospholipids to the triggered by Toll-like binding of various receptor 4 (TLR-4), which plasma proteins could amplify the production of3. a phospholipid-protein autoantibodies. complex is formed and 2. Complement activation a neoepitope is has been increasingly recognized as a possible uncovered significant role in the4. the target of pathogenesis of APS. autoantibodies
ClinicallyClinically, the series of events that leads to hypercoagulabilityand recurrent thrombosis can affect virtually any organ system,including the following:Peripheral venous system (deep venous thrombosis [DVT])Central nervous system (cerebrovascular accident [CVA], sinusthrombosis)Hematologic (thrombocytopenia, hemolytic anemia)Obstetric (pregnancy loss, eclampsia)Pulmonary (pulmonary embolism [PE], pulmonaryhypertension)Dermatologic (livedo reticularis, purpura, infarcts/ulceration)Cardiac (Libman-Sacks valvulopathy, MI)Ocular (amaurosis, retinal thrombosis)Adrenal (infarction/hemorrhage)Musculoskeletal (avascular necrosis of bone)
Epidemiology Frequency: unknown. One to 5% ofhealthy individuals have aPL antibodies.No defined racial predominanceA female predominance has beendocumentedis more common in young to middle-aged adults; however, it also manifests inchildren and elderly people.
Pregnancy morbidityOne or more late-term (>10 weeks gestation)spontaneous abortionsOne or more premature births of a morphologicallyhealthy neonate at or before 34 weeks’ gestationbecause of severe preeclampsia or eclampsia orsevere placental insufficiencyThree or more unexplained, consecutive, spontaneousabortions before 10 weeks’ gestationTh: preferably low–molecular-weight heparin long-term anticoagulation is then continued postpartum. Breastfeeding women may use heparin and warfarin
Laboratory criteria:Patients must have (1) medium to highlevels of immunoglobulin G (IgG) orimmunoglobulin M (IgM) anticardiolipin(aCL), (2) anti–beta-2 glycoprotein I, or(3) LA on at least 2 occasions at least 12weeks apart.
Histologic Findingsnoninflammatory bland thrombosiswith no signs of perivascularinflammation or leukocytoclasticvasculitis
Medical CareProphylactic therapy: CAPS (generally nooral contraceptives, very ill ): smoking, hypertension, or intensive anticoagulation, hyperlipidemia plasma exchange, and Low-dose aspirin corticosteroids (unproven), Clopidogrel Intravenous statins (hyperlipidemia) immunoglobulinThrombosis: Cyclophosphamide (have chemotherapeutic full anticoagulation with activity) (didn’t prove) i/v or s/b heparin + warfarin th Rituximab INR 2.0-3.0 for venous Hydroxychloroquine thrombosis and 3.0 for (Plaquenil) arterial thrombosis
Consultations, recommendations Rheumatologist If warfarin therapy is Hematologist instituted, instruct the Neurologist, cardiologist, patient to avoid pulmonologist, excessive consumption hepatologist, of foods that contain ophthalmologist vitamin K. (depending on clinical Limit activity in patients presentation) with acute DVT. Obstetrician with Instruct the patient to experience in high-risk avoid prolonged pregnancies immobilization.
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