Surgical Treatment of Pulmonary Complications in Behçet's Syndrome

Behcet’s disease (BD) is a rare autoimmune disorder that results in diffuse full-thickness vasculitis. Pulmonary artery aneurysms (PAAs) and hemoptysis are known complications of this disease process, with high morbidity and mortality for affected patients. Although medical, endovascular, and surgical treatment strategies have all been described in the literature, there are little data to describe the long-term outcomes of these various treatment modalities and there continues to be a lack of clearly defined algorithms for the management of these patients. We report a case of PAA in the setting of BD who was treated over the course of many years with medical therapy and coil embolization but ultimately failed treatment, sustained a complication of coil erosion and migration into the trachea twice, and required surgical lobectomy for definitive management. We discuss an algorithm for the management of patients with BD who have PAAs.

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“…Surgery has been used to treat PAAs [14]. Proximal aneurysms may be reconstructed with aneurysmorrhaphy [15,16].…”

Section: Discussionmentioning

confidence: 99%

Pulmonary Artery-Tracheal Fistula After Coil Implantation for Behcet’s Disease

Hansen

Dyke

2020

Cureus

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“…Despite fragility of vascular tissues in Behçet's disease which brings more surgical risks, lobectomies can be done with favorable outcomes. [ 9 ] Due to the fragility of connective tissues, arteries can be tied with tapes and, then, reinforced with transfixion sutures.…”

Section: Discussionmentioning

confidence: 99%

Behçet's disease presenting with massive hemoptysis related to bronchovascular fistula: A case report

Bedirhan¹,

Arda²,

Tanrıverdi³

et al. 2021

Turk Gogus Kalp Dama

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A 37-year-old male patient was admitted to our hospital with recurrent hemoptysis, 50 mL per day. Thoracic computed tomography showed no pathology responsible for hemoptysis. Bronchoscopy revealed mucosal infiltrations and 2 to 3-mm blotch in the lateral wall of the right lower lobe. After punch biopsy of the suspected area, massive bleeding occurred. Right lower bilobectomy was performed urgently. A bronchovascular fistula was noticed at the specimen. Pathological examination result was compatible with clinically suspected Behçet"s disease. The patient was given high-dose steroid and cyclophosphamide treatment and received azathioprine maintenance treatment for 18 months. He has been symptom-free for three-year follow-up.

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“…Bilateral lower lobectomy may be a better treatment option in our case with giant PAAs. In a study on nine patients who underwent surgical treatment, Tuzun et al 14 concluded that four out of six patients who underwent lobectomy were still alive and that lobectomy was an acceptable method in patients who did not respond to medical treatment.…”

Section: Discussionmentioning

confidence: 99%

An Adolescent Presented With Hemoptysis: Pulmonary Artery Aneurysm in Pediatric Behçet’s Disease

et al. 2019

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Behçet's disease (BD) is a rare disease, characterized by chronic perivascular inflammation, recurrent oral and genital ulcers, ocular inflammation and multisystemic involvement due to vasculitis. It is more common in countries along the old Silk Road such as Turkey, Greece, and Israel. The etiology is unknown, while epidemiologic data point to the role of both genetic and environmental factors. 1,2 BD usually presents at third to fourth decades and is rare in children. Vasculitis in BD can affect both arteries and veins; however, venous involvement is more common. Although the most common arterial involvement is pulmonary artery aneurysm (PAA), it occurs very rarely in BD. In a retrospective analysis, the prevalence of pulmonary artery involvement including PAA and pulmonary artery thrombosis was found to be 2%. 3 To our knowledge, there is no data about the prevalence of PAA in children with BD. Patients with PAA usually present with hemoptysis and carry a high risk of mortality. 4 It should be kept in mind that some life-threatening features in the pediatric population may occur earlier than mucocutaneous findings. In this article, we report an adolescent diagnosed with BD to emphasize the importance of vascular etiologies such as BD in the differential diagnosis of hemoptysis even in the pediatric age group.

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Surgical Treatment of Pulmonary Complications in Behçet's Syndrome

Cited by 19 publications

References 26 publications

Pulmonary Artery-Tracheal Fistula After Coil Implantation for Behcet’s Disease

Pulmonary Artery-Tracheal Fistula After Coil Implantation for Behcet’s Disease

Behçet's disease presenting with massive hemoptysis related to bronchovascular fistula: A case report

An Adolescent Presented With Hemoptysis: Pulmonary Artery Aneurysm in Pediatric Behçet’s Disease

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