Treatment of Behçet's Syndrome with Parenchymal Central Nervous System Involvement

Clinical scenario

This protocol addresses patients with Behçet's syndrome who develop parenchymal central nervous system (CNS) involvement — a serious manifestation that requires prompt, structured management distinct from other disease manifestations.

Parenchymal CNS involvement

Parenchymal CNS disease in Behçet's syndrome represents one of the most severe organ manifestations. Acute attacks require urgent intervention with a structured corticosteroid and immunosuppressive strategy. The choice of immunosuppressive agent is not interchangeable — certain agents are contraindicated in this setting.

Treatment approach (partial)

Acute attacks are managed with high-dose intravenous corticosteroids followed by a gradual oral taper, combined with an immunosuppressive agent. The specific regimen, sequencing, and agent selection — including which agents to avoid — are detailed in the full protocol.

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References

DOI: 10.1136/annrheumdis-2018-213225

Acute attacks of parenchymal involvement should be treated with high-dose glucocorticoids followed by slow tapering, together with immunosuppressives such as azathioprine.
A typical glucocorticoid regimen would be starting with daily pulses of intravenous methylprednisolone 1 g/day that may be continued for up to 7 days followed by oral prednisolone (or prednisone) at 1 mg/kg/day for 1 month and tapered by 5-10 mg every 10-15 days.
Cyclosporine-A should be avoided.

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