Pulmonary artery involvement in Behçet's disease patients: real-life data on aneurysm and thrombosis.

摘要

BACKGROUND: Pulmonary vascular involvement represents one of the most severe manifestations of Behçet's disease. Pulmonary artery aneurysm (PAA) and pulmonary artery thrombosis (PAT) are rare but potentially life-threatening complications. However, data regarding their frequency, coexistence, and clinical course remain limited, particularly from a pulmonology-based long-term follow-up perspective. OBJECTIVES: To evaluate the frequency and clinical characteristics of PAA and PAT in a large single-center Behçet's disease cohort and to describe diagnostic and therapeutic challenges encountered during routine clinical practice. DESIGN: Retrospective single-center observational cohort study. METHODS: Adult patients diagnosed with Behçet's disease and followed at a tertiary pulmonology center between January 2014 and January 2024 were retrospectively reviewed. Pulmonary vascular involvement was assessed using contrast-enhanced thoracic computed tomography and CT pulmonary angiography. The presence of deep vein thrombosis (DVT), treatment approaches, and clinical outcomes were recorded. RESULTS: Among 279 patients, pulmonary vascular involvement was identified in 9 patients. Pulmonary artery aneurysm was detected in three patients (1.07%), and pulmonary artery thrombosis in six patients (2.1%). Concomitant DVT was observed in a minority of patients with pulmonary vascular involvement. Clinical follow-up revealed heterogeneous disease courses, including aneurysm development during anticoagulant therapy, hemoptysis-related treatment discontinuation, persistent pulmonary thrombosis, and rare interventional complications such as late coil migration. Mortality occurred in patients with severe pulmonary vascular disease. CONCLUSION: Pulmonary vascular complications in Behçet's disease are rare but associated with significant morbidity and mortality. The coexistence of inflammatory and thrombotic processes creates substantial diagnostic and therapeutic challenges, particularly regarding anticoagulation strategies. Although the limited number of cases precludes definitive conclusions regarding pathogenetic mechanisms, our findings highlight the need for careful, individualized, and multidisciplinary management of pulmonary vascular involvement in Behçet's disease. Pulmonary Blood Vessel Problems in Behcet's Disease: A 10-Year ExperienceBehcet's disease is a chronic inflammatory condition that can affect blood vessels throughout the body. Although involvement of the lungs is uncommon, problems affecting the blood vessels in the lungs can be serious and sometimes life-threatening. These problems may include abnormal widening of the pulmonary arteries or the formation of blood clots within these vessels. In this study, we reviewed the medical records of patients with Behcet’s disease who were followed at our center over a 10-year period. Our aim was to better understand how often lung blood vessel problems occur, how they present clinically, and how they are managed in real-life practice. We also evaluated blood clots in the legs, which are common in Behcet’s disease and may coexist with lung involvement. We found that lung blood vessel complications were rare but associated with significant clinical challenges, including bleeding, treatment-related risks, and, in some cases, death. The clinical course varied considerably between patients, highlighting that there is no single approach suitable for all cases. Treatment decisions, particularly regarding the use of blood thinners, were often complex and had to be individualized. Our findings emphasize the importance of careful evaluation of lung symptoms in patients with Behcet’s disease and the need for close collaboration between different medical specialties. Early recognition and personalized management may help reduce serious complications and improve patient outcomes.