Antiphospholipid syndrome

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在2018年3月13日 (二) 17:47所做的修訂版本 (編輯)
Guhjy (對話 | 貢獻)
(新頁面: *Antibody to anionic phospholipids, particularly ''β2 glycoprotein 1'' *Diagnostic criteria **Presence in the serum of at least one type of autoantibody known as an antiphospholipid ...)
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當前修訂版本 (2018年3月13日 (二) 17:47) (編輯) (撤銷)
Guhjy (對話 | 貢獻)
(新頁面: *Antibody to anionic phospholipids, particularly ''β2 glycoprotein 1'' *Diagnostic criteria **Presence in the serum of at least one type of autoantibody known as an antiphospholipid ...)
 

當前修訂版本

  • Antibody to anionic phospholipids, particularly β2 glycoprotein 1
  • Diagnostic criteria
    • Presence in the serum of at least one type of autoantibody known as an antiphospholipid antibody, which is directed against phospholipid-binding plasma proteins
    • The occurrence of at least one clinical feature from a diverse list of potential disease manifestations, the most common of which are categorized as venous or arterial thromboses, pregnancy morbidity, or thrombocytopenia
  • Idiopathic, pregnancy, hemodialysis, RT, GN (minimal change disease, membranous nephropathy), SLE (lupus anticoagulant with activated partial tissue thromboplastin time ↑)
  • Clinical features
    • Hematuria, proteinuria
    • Vasculitis
      • Treated by corticosteroids
    • Severe form: hemolytic anemia, deep vein thrombosis, transient ischemic attacks, pulmonary embolism, spontaneous abortions
      • <10% present have catastrophic multi-organ involvement and acute renal failure
      • Patients who present with acute flank pain and renal vein thrombosis in the setting of proteinuria should always be checked for antiphospholipid antibodies
    • Less severe form
    • P'ts can wax and wane, and many patients have recurrences
  • Thrombotic microangiopathy of the kidneys (25%)
    • Affects interlobular artery, arterioles and glomerular capillaries
    • Microthrombi
    • Endotheliosis (also seen in preeclampsia/HELLP [hemolysis, elevated liver enzymes, and low platelet count syndrome], malignant hypertension)
    • Treatment
      • Warfarin
      • Plasmapheresis and immunosuppresives for acute renal failure