Antiphospholipid syndrome
出自KMU Wiki
- 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
