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. |