Childhood Primary Central Nervous System Vascultis Treatment Protocols
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1 Childhood Primary Central Nervous System Vascultis Treatment Protocols Last updated December 2014
2 Non-progressive large vessel primary CNS vasculitis* Adjunctive immunosuppression f 3 months IV Methylprednisolone 30mg/kg (max 1g) x days (3 to 5) Month 1 Start date x 3 months tapering monthly e.g _ mg _ mg Month 2 _ mg Month 3 Repeat Vascular Imaging Progression: new vascular stenosis new territies involved Progression on imaging Rapid taper Weekly taper e.g stop Begin progressive cpacns protocol Optional: Acyclovir (80 mg/kg/day PO divided QID, consider renal adjustment) x days (5 to 14, if indicated) * Includes entities of: Angiography-positive, non-progressive primary CNS vasculitis (NPcPACNS), Transient cerebral arteriopathy (TCA), Focal cerebral arteriopathy (FCA), and Post-varicella angiopathy (PVA)
3 Progressive primary large vessel CNS vasculitis* Phase 1: Induction Therapy (0-6 months) IV Methylprednisolone pulses 30mg/kg (max 1g) x days (3-7) Start date tapering monthly** mg mg mg mg mg mg mg Cyclophosphamide ( mg/m 2 monthly x 7 doses) Trimethoprim/sulfamethoxazole (PJP prophylaxis dosing) *Angiography-positive, progressive primary CNS vasculitis (PcPACNS) 1
4 Progressive primary large vessel CNS vasculitis* Phase 2: Maintenance Therapy (7-18 months) Tapering monthly** mg Choose: End of taper date Mycophenolate mofetil ( mg/m 2 divided BID) Dose: Mycophenolic acid ( mg/m 2 divided BID) Dose: Azathioprine (2-3 mg/kg, max 150 mg, PO daily) Dose: *Angiography-positive, progressive primary CNS vasculitis (PcPACNS) 2
5 Primary small vessel CNS vasculitis* Phase 1: Induction Therapy (0-6 months) IV Methylprednisolone pulses 30mg/kg (max 1g) x days (3-7) Start date tapering monthly** mg mg mg mg mg mg mg Cyclophosphamide ( mg/m 2 monthly x 7 doses) Trimethoprim/sulfamethoxazole (prophylaxis dosing) *Angiography-negative, brain-biopsy positive small vessel primary CNS vasculitis (SVcPACNS) 1
6 Primary small vessel CNS vasculitis* Phase 2: Maintenance Therapy (6-18 months) Tapering monthly** mg Choose: Mycophenolate mofetil ( mg/m 2 divided BID) Dose: Mycophenolic acid ( mg/m 2 divided BID) Dose: Azathioprine (2-3 mg/kg, max 150 mg, PO daily) Dose: *Angiography-negative, small vessel primary CNS vasculitis (SVcPACNS) 2
7 Refracty primary CNS vasculitis* Phase 1: Induction 0-6 months tapering biweekly** Infliximab (5-7.5 mg/kg IV, at diagnosis and after 2 weeks, then monthly) IVIG ( mg/m 2 monthly) Methotrexate (10-15 mg PO/subcutaneously weekly) Dose: Folic Acid (1 mg/day PO) * Failure to respond to induction therapy 1
8 Refracty primary CNS vasculitis* Phase 2: Maintenance (7-12 months) Infliximab (5-7.5 mg/kg IV monthly) Optional: IVIG ( mg/m 2 monthly) Methotrexate (10-15 mg PO/subcutaneously weekly) Dose: Folic Acid (1 mg/day PO) * Failure to respond to induction therapy 2
9 Disease activity flare of primary CNS vasculitis* Treat f 6 months Min flare tapering biweekly** IVIG ( mg/m 2 monthly) Mycophenolate mofetil ( mg/m 2 divided BID) Dose: Mycophenolic acid ( mg/m 2 divided BID) Dose: Maj flare Choose as per clinical presentation: Repeat Phase 1: Induction Therapy protocol Proceed to Refracty Primary CNS Vasculitis Therapy protocol * Defined as recurrence of symptoms relative to baseline functional status 1
Childhood Primary Central Nervous System Vascultis Treatment Protocols
Childhood Primary Central Nervous System Vascultis Treatment Protocols Last updated November 2015 Non-progressive large vessel primary CNS vasculitis* Adjunctive immunosuppression f 3 months IV Methylprednisolone
RATIONALE. K Without therapy, ANCA vasculitis with GN is associated. K There is high-quality evidence for treatment with
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