中枢神经系统血管炎课件.ppt

MELAS DNA testing MELAS 3243-tRNA leu 3243G MELAS 3271-tRNA leu T3271C MELAS 3252-tRNA leu A3252G MELAS 3256-tRNA leu C3256T MELAS 3291-tRNA leu T3291C MELAS 13,513-ND5 G13513A Treatment for CNS vasculitis CYTOXIC AGENT CORTICOSTEROIDS _____________________________________________ Induction Cyclophosphamide 2mg/kd daily Prednisolone 1mg/kg daily therpay by mouth (max 150mg); lower (max 80mg); Reduce weekly to 4 – 6 mo dose by 25mg if 60 years WBC 10mg/day by 6 months must be 4.0 x 10 /1 Maintenance Azathioprine 2mg/kg daily Prednisolone 5 - 10mg/day therapy 6 – 24 mo MTX Escalation Acute severe disease with creatinine 500 umol/1 or pulmonary therapy hemorrhage; Consider 7 – 10 plasma exchange treatment over 14 days such that 60 ml/kg of plasma is exchanged for 4.5% or 5% human albumin solution or consider three pulses of methylprednisolone, 15 mg/kg daily for 3 days. These patients (if under 60 years) may also require 2.5mg/kg daily of cyclophosphamide. Thank you! Central Nervous System Vasculitis James Jinxing Wang, MD, PhD Clinical Assistant Professor University of TN Memphis, TN jjxwang@ Classification of CNS vasculitis INFECTIOUS VASCULITIS -- Spirochetal (syphilis) -- Mycobacterial -- Fungal -- Rickettsial -- Bacterial (purulent) meningitis -- Viral -- Other organisms NECROTIZING VASCULITIDES -- Classic polyarteritis nodosa -- Wegener’s granulomatosis -- Allergic Angitis and granulomatosis (Churg-Strauss) -- Necrotizing systemic vasculitis-overlap syndrome -- Lymphomatoid granulomatosis VASCULITIS ASSOCIATED WITH COLLAGEN VASCULAR DISEASES -- Systemic lupus erythematosus -- Rheumatoid arthritis -- Scleroderma -- Sjogren’s syndrome GIANT CELL ARTERITIDES -- Takayasu’s arteritis -- Temporal (cranial) arteri