Behçet’s Disease: Pulmonary Aneurysm Resolution with Oral Therapy and HLA B72 Allele Association

Abstract

We report the case of a woman who was diagnosed with a pulmonary artery aneurysm that was caused by Behçet's disease. The patient was initially diagnosed with community-acquired pneumonia and then pulmonary thromboembolism and aneurysm of the right pulmonary artery segmental branch was confirmed. The initial treatment consisted of anticoagulant drugs. After analysis of the family history and a positive pathergy test, the patient was diagnosed with Behçet's disease. Oral pharmacological treatment began with corticosteroids, cyclophosphamide, and anticoagulant suspension. The HLA B72 allele was identified in the patient and her two sisters, demonstrating the familial characteristic of the disease and the presence of this allele in a female patient with Behçet's disease. After 12 months of treatment, the clinical condition completely resolved.

Vanishing aneurysms in Behcet’s disease - A case series

“…The etiopathogenesis of BD is unspecified, however various associations with human leukocyte antigen HLA-B51, genetic mutations including factor V Leiden, and multiple other discrete alleles of HLA have been identified [6][7][8]. Inflammation of the vasa-vasorum of the tunica media layer results in the destruction of elastic fibers of media with consequent development of vessel lumen dilatation and subsequent aneurysm formation [9].…”