Antiphospholipid syndrome

出自KMU Wiki

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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