Surgical Treatment of Pulmonary Complications in Behçet's Syndrome

Abstract

We described disease characteristics, management and outcome of a group of Behçet's syndrome (BS) patients who underwent pulmonary lobectomy, segmentectomy or various pleura interventions for complications due to pulmonary artery or descending aorta involvement. There were 9 patients with BS (8 M/1 F; mean age: 24.8 ± 7.5 and mean disease duration: 4.3 ± 3.8 years) who underwent lung surgery from 2000 to 2017. Their medical files including operation details and radiological and pathological studies were reviewed retrospectively. Lobectomy was done in 6 patients, decortications and pleura interventions in the remaining 3. The reason for the surgical procedures were giant pulmonary arterial aneurysms refractory to the medical treatment (n = 4), pneumothorax due to large cavities (n = 2), pleural effusions refractory to the medical treatment (n = 1), a bronchopleural fistula after embolization (n = 1) and bronchiectasis (n = 1). All patients received medical treatment with cyclophosphamide and corticosteroids before the surgical interventions. The histological examination in patients with pseudo-aneurysms showed destruction of medial elastic fibers, inflammatory cell infiltration of adventitial tissues and penetration into the adjacent bronchi with adherent thrombi. Two patients died: one due to Budd-Chiari syndrome 1 year after the surgery, another because of a massive hemoptysis 3 months after lobectomy. The remaining 7 patients are still alive with a median follow-up of 8 years (inter-quartile range [IQR]: 4-11). Lobectomies in BS patients with giant pulmonary aneurysms refractory to medical treatment can be done with favorable outcome. Also, complications such as large cavities causing pneumothorax, refractory pleural effusions, bronchiectasis, and embolization complications can be managed effectively with surgical interventions.