CC May 2017 Antiphospholipid Antibody Syndrome

echoecho's version from 2017-05-15 12:59


Question Answer
Define Antiphospholipid antibody syndrome? It is an immune disorder characterized by the presence of antibodies in the blood directed against phospholipids
Diagnosis of APS requires both ____ and ____ evidence of the disease?1) clinical 2)laboratory
What two complications can occur as a result of APS?1)thrombotic 2)obstetric
*** APS is associated with both ____ and ____ thrombosis?1) arterial 2)venous
*** List the 4 obstetric complication?1) recurrent (3 or more) unexplained early pregnancy losses 2)unexplained fetal demise after 10 weeks'gestation 3)placental insufficiency 4) severe pre-eclampsia
*** Diagnosis requires the detection of specified autoantibodies on ____ occasions at least ___ weeks apart?2; 12
The diagnosis can be satisfied by a positive _______ for IgG or IgM autoantibodies directed against_______, against _______ or by demonstration of _____ anticoagulant activity?1) ELISA 2) cardiolipin 3) beta-2-glycoprotein 1 4) lupus anticoagulant
Define lupus anticoagulant activity?abnormal prolongation of in vitro clotting times that is NOT corrected by adding serum containing normal clotting factor leaves
This effect may be caused by the ______ previously noted or by other autoantibodies directed against ________of the hemostatic system? 1) autoantibodies 2) components
Patients with a triple positive antibody profile (anti-beta2-glycoproteins I, anticoardiolipin and lupus anticoagulant activity) have a higher _____ risk than those with only a single lab abnormality? thrombotic
Though Antiphospholipid syndrome may be associated with SLE or other autoimmune diseases, primary Antiphospholipid syndrome occurs in the absence of what?other autoimmune disease
The pathological autoantibodies may exert their effect via several mechanisms, list these?1) disrupting the normal balance between the clotting and fibrinolytic systems and the autoantibodies may cause direct endothelial injury, leading to endothelial hyperplasia 2) Antiphospholipid antibodies may disrupt the normal process by which the trophoblast INVADES the vessels of the placenta leading to placental insufficiency and obstetric complications
*** What is the significance of a positive lab test in the absence of a hx of a thrombotic or obstetric complication?it is unclear
*** Patients should be selected for testing based on what?their protest probabilityof disease
*** Testing is most appropriate in patients with what?1) unprovoked thrombosis before age 50 2) thrombosis at unusual sites 3) late or recurrent pregnancy losses 4) any thrombotic or obstetric complications in the setting of known autoimmune disease
Positive testing requires confirmation after ____ weeks?12
Appropriateness of testing is low in what clinical presentation?venous or arterial thromboembolism in elderly patients
Appropriateness of testing is moderate in what clinical presentations?1) incidentally noted prolonged activated partial thromboplastin time (aPTT) 2)recurrent spontaneous early pregnancy loss 3)provoked venous thromboembolism in young patients
Appropriateness of testing is high in what clinical presentations?1) unprovoked / unexplained venous or arterial thrombosis in young patients (<50 years) 2) thrombosis at unusual sites 3)late pregnancy loss 4) thrombosis or obstetric morbidity in patients with known autoimmune disease
*** Nonpregnant patients with Antiphospholipid syndrome AND a hx of thrombosis should be generally be treated for life with what?vitamin K antagonist such as Warfarin
What is the goal for INR?2.5-3.5
Pregnant patients should be treated with what?1) low-dose ASA AND 2) either unfractionated or low-molecular-weight heparin
Some experts recommend _______ low-dose ASA therapy for patients with obstetric Antiphospholipid syndrome?life-long
Patients with Antiphospholipid antibodies but WITHOUT clinical events can be treated with what?ASA 81 mg daily
What is the potential role of use of NOACs (novel oral anticoagulants?some case series have found high rates of recurrent thormbosis while other cases have found acceptable effectiveness
What is the Global Antiphospholipid Syndrome Score?it is a clinical scoring system to stratify patients based on future thrombotic risk. It has been validated both in patients with primary Antiphospholipid syndrome and puts with underlying SLE. The score incorporates both traditional cardiovasculr risk factors and specifi autoantibody test
In a population of patients with primary Antiphospholipid syndrome, a Global Antiphospholipid Syndrome Score > 11 was ___% sensitive and ___% specific for identifying patients with recurrent thrombosis?94%; 78%
Is the GAPSS (Global Antiphospholipid Syndrome Score) currently included in formal treatment guidelines? If not, what can it be used for?1) no 2) clinical decision-making for patients
List the Global Antiphospholipid Syndrome Score as to clinical finding and point value?1) hyperlipidemia = 3 points; 2) HTN = 1 point; 3) Anticardiolipin IgG or IgM = 5 points; 4) anti-beta2-glycoproteins 1 IgG or IgM = 4 points; 5) Anti-phosphatidylserine-prothrombin complex = 3 points; 6)lupus anticoagulant = 4 points
Regarding the GAPSS, a higher score indicates what?a greater risk of thrombosis
Although not currently incorporated into treatment recommendations, what two agents may have a role in treating Antiphospholipid syndrome?1) immunosuppressant agents 2) anti-inflammatory agents
What medication is recommended for patients with SLE and Antiphospholipid antibodies and limited data suggest that this drug can benefit Antiphospholipid syndrome as well?Hydroxychloroquine
Limited data suggest that ____ and _____ may have a role in reducing inflammation and thrombosis?1) statins. 2) rituximab
*** SUMMARY = Antiphospholipid antibody syndrome (APS) requires both ____ and ____ evidence for diagnosis?clinical; lab
*** SUMMARY = What medication treated for life is recommended for nonpregnant patients with APS and clinical manifestations of thrombosis?Vit K antagonists
*** SUMMARY = What is the INR goal for the above?2.5 -3.5
*** SUMMARY = What is the role of novel anticoagulants in APS?unclear and they are NOT recommended for use in APS patients