Treatment of Behçet's Disease with Pulmonary Artery Aneurysms

Pulmonary artery aneurysms are a serious vascular complication of Behçet's syndrome and require urgent, structured management. This protocol addresses the specific approach recommended for this presentation.

Clinical Scenario Behçet's syndrome with pulmonary artery aneurysms — a high-risk vascular manifestation requiring prompt escalation of therapy beyond standard Behçet's management.

Treatment Approach (partial) Management centres on high-dose glucocorticoids in combination with an immunosuppressive agent. For patients at elevated risk of major bleeding, a vascular intervention approach is recommended over open surgery. Full regimen, sequencing, and vascular management details are available in the complete protocol.

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References

DOI: 10.1136/annrheumdis-2018-213225

For the management of pulmonary artery aneurysms, high-dose glucocorticoids and cyclophosphamide are recommended.
Cyclophosphamide may be given as monthly intravenous pulses and glucocorticoids are usually given as three successive intravenous methylprednisolone pulses followed by oral prednisolone (or prednisone) at a dose of 1 mg/kg/day.
For patients who have or who are at high risk of major bleeding, embolisation should be preferred to open surgery.

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