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Anti phospholipid antibody syndrome

Anti phospholipid antibody syndrome. Pulmonary. Pulmonary hypertension ‡ Diffuse alveolar hemorrhage † Acute respiratory distress syndrome †. Cardiovascular. Myocardial infarction Accelerated atherosclerosis Heart valve lesions † Syndrome X Deep vein thrombosis. CNS. Strokes

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Anti phospholipid antibody syndrome

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  1. Anti phospholipid antibody syndrome

  2. Pulmonary • Pulmonary hypertension‡ • Diffuse alveolar hemorrhage† • Acute respiratory distress syndrome†

  3. Cardiovascular • Myocardial infarction • Accelerated atherosclerosis • Heart valve lesions† • Syndrome X • Deep vein thrombosis

  4. CNS • Strokes • Transient ischemic attacks • Chorea and movement disorders† • Epilepsy† • Transverse myelitis† • Multiple sclerosis-like disease† • Memory loss†

  5. Ophthalmic  • Visual problems (CRVO, CRAO, PRVO and PRAO)

  6. Ear nose throat • Sudden sensorineural hearing loss • Balance disturbances

  7. Blood • Thrombocytopenia

  8. Endocrine • Adrenal insufficiency • Pituitary infarction

  9. Orthopedic  • Osteonecrosis • Avascular necrosis

  10. Renal  • RAS and renal HTN • Renal vein thrombosis • Renal failure†

  11. Gastroenterology • Mesenteric ischemia (acute-chronic) • Pancreatitis • Peptic ulcer disease • Bowel ischemia and perforation • Budd-Chiari syndrome

  12. Reproductive (obstetric) • Spontaneous abortions • Fetal loss† • Premature births • Fetal growth restriction • Pre-eclampsia

  13. Asymptomatic aPL-positive patients† • High thrombosis risk:- Lifestyle modifications and aspirin/hydroxychloroquine • Low thrombosis risk:- Lifestyle modifications only

  14. Secondary thrombosis prevention • Index venous event:- Lifelong anticoagulation with INR (2-3) • Index arterial: event/recurrent event- Lifelong anticoagulation with INR (3-4) §

  15. Obstetric APS patients • Clinical criteria vascular:- Major/recent event: aspirin and therapeutic dose UFH/LMWH- Minor/remote event: aspirin and prophylactic dose UFH/LMWH • Clinical criteria obstetric:- Aspirin and prophylactic dose UFH/LMWH

  16. Catastrophic APS • Life threatening:- Anticoagulation, intravenous corticosteroids, and IVIG and/or plasma exchange • Stable:- Anticoagulation and intravenous corticosteroids, ± IVIG and/or plasma exchange

  17. Noncriteria manifestations • Thrombocytopenia (clinically significant):- Corticosteroids, rituximab, IVIG or splenectomy • Cardiac valvular disease:- Anticoagulation (in the presence of atrial fibrillation or other thrombosis risk factors) • Nephropathy:- Anticoagulation and angiotensin-converting enzyme inhibitor • Cognitive dysfunction:- No available therapeutic intervention

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