Background Behcet's disease (BD) is a chronic inflammatory disorder. Arterial inflammatory involvement includes predominantly aortic and pulmonary aneurysmal lesions, affects about 10% of patients with BD. They account for the severity of the disease and are a leading cause of death
Objectives To investigate the frequency of Behcet's disease with pulmonary artery aneurysm (PAA). We aimed to review PAA and other systemic involvements associated with PAA in BD and to provide a review of diagnostic techniques, treatment and prognosis
Methods 243 BD patients were recruited for this study (152 men, 91 women, mean age 31.7±7 years. Diagnosis of BD was made according to the international study group for Behçet's disease [International Study Group for Behçet's Disease, lancet 1990; 335: 1078–80]. All patients underwent full clinical examination, routine laboratory investigations. Chest X-rays and pulmonary CT angiography were performed on all patients with pulmonary involvement.
Results Eight of the patients have pulmonary aneurysm, all of them are male, mean age 32.6±13, The mean disease duration until PAA appear was 2.8±3.5 years. The main pulmonary symptoms were as follows: dyspnea 87%, cough 50%, hemoptosis: 75%, fever 37%. Other systemic involvements associated PAA are as follows: buccal (100%) and genital (75%) ophthalmic 25%, neurological 50%, cardiac 25%. 3 patients presented with Hughes Stovin syndrome. The treatment includes corticosteroids, colchicine and immunosuppressant agents (Cyclophosphamid or azathioprine), only two patients reveive coil embolization. At follow up for a median of 4 years (1 to 25 years), three patients died because they stopped their medication.
Conclusions The prognosis of PAA is poorer than other lesions involved in BD, treatment (immunosuppressant agents, colchicine) seems to improve the prognosis. It is important to maintain the immunosuppressive therapy and a regular follow-up to prevent these complications
Desbois AC et al Aortic inflammatory lesions in Behçet's disease Rev Med Interne. 2016 Apr;37(4):230–8.
Celik S et al. Pulmonary artery aneurysms in Behçet's syndrome: a review of the literature with emphasis on geographical differences. Clin Exp Rheumatol. 2015 Nov-Dec;33(6 Suppl 94):S54–9.
Disclosure of Interest None declared
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