This protocol covers Behçet’s syndrome presenting with pulmonary artery aneurysms — a serious vascular manifestation that defines a distinct clinical subgroup requiring a targeted management approach.
Pulmonary artery aneurysms represent one of the most severe vascular complications in Behçet’s syndrome. Their presence calls for specific intervention that differs from the general management of the disease.
For the management of pulmonary artery aneurysms, high-dose glucocorticoids and cyclophosphamide are recommended.
Monoclonal anti-TNF antibodies should be considered in refractory cases.
Observational, uncontrolled evidence showed that infliximab provided benefit in some of the refractory patients.
DOI: 10.1136/annrheumdis-2018-213225 View source ↗