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level: Level 1 of Ch16: Antiphospholipid Syndrome

Questions and Answers List

level questions: Level 1 of Ch16: Antiphospholipid Syndrome

QuestionAnswer
What is APS?thrombophilic disorder, specific groups of autoantiboides called aPL, arterial or venous thrombosis/ pregnancy morbidity, may be primary or secondary. Autoantibody found is lupus anticoagulant by a coagulation based test, anti cardiolipine IgG/IgM by ELISA, b 2 glycoprotein I. Clinical events seen are vascular thrombosis and pregnancy complications. At least 1 clinical event with 1of 3 autoantibodies are the dx.
What are conditions associated with aPL?CT disorders (SLE, RA, SS, SSc, Myositis), Drugs, Crohns, Systemic vasculitis, Infections, malignancy, other.
What are the possible consequences of APS?Cardiovascular (CVA, sinus thrombosis, cardiac valvupathy, MI, DVT in vein, intracranial thrombos arterial, CAD) Dermatologic (livedo reticularis [poor prognosis irresponsive to tx], purpura, infarct/ulcer) Hemato (thrombocytopenia, hemolyic anemia, thromboembolism) Musculoskeletal (avascular necrosis of bone /infarction/ hemorrhage) Neuro (stroke, ischemic attack, cognitive dysfunction, migraine R/O MS) OBGYN (spontaneous abortion, preterm delivery, preeclampsia, placental insufficiency) Ophthalmo (ocular thrombosis) Pulmo (embolism, pulmo HTN) Kidney (R/O SLE (IC) vs APS (thrombosis), liver and GI (liver T) CAPS (accelerated APS multiorgan failure over short time typically days to weeks, very poor prognosis)
What are the criteria of APS?Sydney
What are criteria of diagnosis of CAPS?Involvement of 3 or more organs/tissues, development in less than 1 week, confirmed by histopathology small vessel occlusion in at least one organ or tissue, labs presence of aPL (LA/aCL/Anti-b2GPI) Definite catastrophic APS all 4 criteria.