BACKGROUND: Arterial involvement is a rare but serious condition in the course of Behçet's disease. We aimed to assess the results of therapeutic approaches in our patients with arterial lesions caused by Behçet's disease. PATIENTS AND METHODS: The records of 534 patients with Behçet's disease between 1987 and 2002 were retrospectively evaluated for the presence of arterial lesions. All patients were followed up regularly at 3 to 6 months intervals. RESULTS: Arterial lesions were diagnosed in 21 (3.9%) patients. Eight of these patients had pulmonary artery aneurysms (PAA), and the other 13 patients had non-pulmonary arterial lesions. Urgent surgical intervention was performed in three patients with PAA leading to death in all three. In addition, three other patients died due to massive haemoptysis at home despite to immunosuppressive therapy. Only two out of eight patients with PAA are still alive who were treated with cyclophophamide and corticosteroids. Thirteen operations were performed in 7 out of 13 patients having non-pulmonary arterial lesions. Although ten of the operations were primary operations, three reoperations had to be performed. A stent-graft was applied for the management of an iliac artery aneurysm in one patient. Only one patient died 8 years after the first non-pulmonary arterial involvement following a type IV thoracoabdominal aortic aneurysm repair. Five patients with arterial occlusive lesions were successfully treated by corticosteroids. CONCLUSIONS: Pulmonary artery aneurysms in Behçet's disease patients have a poor prognosis despite any form of therapy. High dose corticosteroids alone can be successfully used for isolated non-pulmonary arterial occlusive lesions, unless disabling symptoms occur. Surgery or stent-graft insertion is indicated for non-pulmonary arterial aneurysms because these aneurysms entail high risk of complications.
BACKGROUND: Arterial involvement is a rare but serious condition in the course of Behçet's disease. We aimed to assess the results of therapeutic approaches in our patients with arterial lesions caused by Behçet's disease. PATIENTS AND METHODS: The records of 534 patients with Behçet's disease between 1987 and 2002 were retrospectively evaluated for the presence of arterial lesions. All patients were followed up regularly at 3 to 6 months intervals. RESULTS: Arterial lesions were diagnosed in 21 (3.9%) patients. Eight of these patients had pulmonary artery aneurysms (PAA), and the other 13 patients had non-pulmonary arterial lesions. Urgent surgical intervention was performed in three patients with PAA leading to death in all three. In addition, three other patients died due to massive haemoptysis at home despite to immunosuppressive therapy. Only two out of eight patients with PAA are still alive who were treated with cyclophophamide and corticosteroids. Thirteen operations were performed in 7 out of 13 patients having non-pulmonary arterial lesions. Although ten of the operations were primary operations, three reoperations had to be performed. A stent-graft was applied for the management of an iliac artery aneurysm in one patient. Only one patient died 8 years after the first non-pulmonary arterial involvement following a type IV thoracoabdominal aortic aneurysm repair. Five patients with arterial occlusive lesions were successfully treated by corticosteroids. CONCLUSIONS:Pulmonary artery aneurysms in Behçet's disease patients have a poor prognosis despite any form of therapy. High dose corticosteroids alone can be successfully used for isolated non-pulmonary arterial occlusive lesions, unless disabling symptoms occur. Surgery or stent-graft insertion is indicated for non-pulmonary arterial aneurysms because these aneurysms entail high risk of complications.
Authors: Ahmed Mousa; Ibrahim Hanbal; Alaa Sharabi; Mohammed A Nasr; Abdelfattah K Nassar; Mai A Elkalla Journal: Orphanet J Rare Dis Date: 2019-04-15 Impact factor: 4.123