Childhood Primary Central Nervous System Vascultis Treatment Protocols
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1 Childhood Primary Central Nervous System Vascultis Treatment Protocols Last updated November 2015
2 Non-progressive large vessel primary CNS vasculitis* Adjunctive immunosuppression f 3 months IV Methylprednisolone 30/kg (max 1g) x days (3 to 5) Month 1 2 /kg (max 60g) x 3 months tapering monthly e.g Month 2 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 Calcium (1000 /day PO) Optional: Acyclovir (80 /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 30/kg (max 1g) x days (3-7) 2 /kg (max 60g) tapering monthly** Cyclophosphamide ( /m 2 monthly x 7 doses) Trimethoprim/sulfamethoxazole (PJP prophylaxis dosing) Calcium (1000 /day PO) *Angiography-positive, progressive primary CNS vasculitis (PcPACNS) ** Example prednisone taper schedule: 60 /day, 50, 40, 30, 25, 20, 17.5, 15, 12.5, 10, 7.5, 5, 2.5, stop 1
4 Progressive primary large vessel CNS vasculitis* Phase 2: Maintenance Therapy (7-18 months) Tapering monthly** Choose: End of taper date Mycophenolate mofetil ( /m 2 divided BID) : Mycophenolic acid ( /m 2 divided BID) : Azathioprine (2-3 /kg, max 150, PO daily) : Calcium (1000 /day PO) *Angiography-positive, progressive primary CNS vasculitis (PcPACNS) ** Example prednisone taper schedule: 60 /day, 50, 40, 30, 25, 20, 17.5, 15, 12.5, 10, 7.5, 5, 2.5, stop 2
5 Primary small vessel CNS vasculitis* Phase 1: Induction Therapy (0-6 months) IV Methylprednisolone pulses 30/kg (max 1g) x days (3-7) 2 /kg (max 60g) tapering monthly** Cyclophosphamide ( /m 2 monthly x 7 doses) Trimethoprim/sulfamethoxazole (prophylaxis dosing) Calcium (1000 /day PO) *Angiography-negative, brain-biopsy positive small vessel primary CNS vasculitis (SVcPACNS) ** Example prednisone taper schedule: 60 /day, 50, 40, 30, 25, 20, 17.5, 15, 12.5, 10, 7.5, 5, 2.5, stop 1
6 Primary small vessel CNS vasculitis* Phase 2: Maintenance Therapy (6-18 months) Tapering monthly** Choose: Mycophenolate mofetil ( /m 2 divided BID) : Mycophenolic acid ( /m 2 divided BID) : Azathioprine (2-3 /kg, max 150, PO daily) : Calcium (1000 /day PO) *Angiography-negative, small vessel primary CNS vasculitis (SVcPACNS) ** Example prednisone taper schedule: 60 /day, 50, 40, 30, 25, 20, 17.5, 15, 12.5, 10, 7.5, 5, 2.5, stop 2
7 Refracty primary CNS vasculitis* Phase 1: Induction 0-6 months 2 /kg (max 60g) tapering biweekly** Infliximab (5-7.5 /kg IV, at diagnosis and after 2 weeks, then monthly) IVIG (2g/kg max 70g, monthly) Methotrexate (10-15 /m 2 PO/subcutaneously weekly) : Calcium (1000 /day PO) Folic Acid (1 /day PO) * Failure to respond to induction therapy ** Example prednisone taper schedule: 60 /day, 50, 40, 30, 25, 20, 17.5, 15, 12.5, 10, 7.5, 5, 2.5, stop 1
8 Refracty primary CNS vasculitis* Phase 2: Maintenance (7-12 months) Infliximab (5-7.5 /kg IV monthly) Optional: IVIG (2g/kg max 70g, monthly) ) Methotrexate (10-15 /m 2 PO/subcutaneously weekly) : Calcium (1000 /day PO) Folic Acid (1 /day PO) * Failure to respond to induction therapy ** Example prednisone taper schedule: 60 /day, 50, 40, 30, 25, 20, 17.5, 15, 12.5, 10, 7.5, 5, 2.5, stop 2
9 Disease activity flare of primary CNS vasculitis* Treat f 6 months Min flare 2 /kg (max 60g) tapering biweekly** IVIG (2g/kg max 70g, monthly) Mycophenolate mofetil ( /m 2 divided BID) : Mycophenolic acid ( /m 2 divided BID) : Calcium (1000 /day PO) 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 ** Example prednisone taper schedule: 60 /day, 50, 40, 30, 25, 20, 17.5, 15, 12.5, 10, 7.5, 5, 2.5, stop 1
Childhood Primary Central Nervous System Vascultis Treatment Protocols
Childhood Primary Central Nervous System Vascultis Treatment Protocols Last updated December 2014 Non-progressive large vessel primary CNS vasculitis* Adjunctive immunosuppression f 3 months IV Methylprednisolone
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