CHEST Guidelines-Recurrent-Pulmonary-Aneurysms

Recurrent-Pulmonary-Aneurysms_chest

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The document presents a detailed case study of a 34-year-old South African man with recurrent life-threatening hemoptysis due to multiple pulmonary aneurysms, ultimately diagnosed as Behçet disease with strong ties to Hughes-Stovin syndrome. Initially, the patient presented with eye issues showing panuveitis and was lost to follow-up after treatment. Two years later, he experienced massive hemoptysis, precipitated by pulmonary artery aneurysms and extensive venous thrombosis, leading to the diagnosis of Hughes-Stovin syndrome. Initial treatments included pulmonary artery coil embolization and a challenging right lower lobectomy, which provided limited relief.<br /><br />After recurrent symptoms, further investigations revealed thrombosis extending from the femoral veins to the inferior vena cava. Treatments with prednisone and azathioprine showed positive outcomes, with the eventual resolution of aneurysms and relief from hemoptysis.<br /><br />The case emphasizes the complexity of distinguishing between Hughes-Stovin syndrome, characterized by pulmonary artery aneurysms and venous thrombosis, and Behçet disease, which shows similar vascular involvement. The diagnosis of Behçet disease was supported by previously recorded ocular symptoms, oral ulceration history, and a positive pathergy test.<br /><br />Discussion highlights the diagnostic and therapeutic challenges in managing conditions involving both extensive thrombosis and bleeding in Behçet disease. The report questions whether Hughes-Stovin syndrome should be considered a distinct clinical entity or a subset under the Behçet disease spectrum due to overlapping clinical features.<br /><br />This report underlines the need for comprehensive evaluations and targeted immunomodulatory treatments in managing complex vasculitic disorders and reinforces the convoluted nature of vascular involvement across different syndromes within the Behçet disease spectrum.

Keywords

Behçet disease

Hughes-Stovin syndrome

pulmonary aneurysms

hemoptysis

panuveitis

venous thrombosis

pulmonary artery embolization

immunomodulatory treatments

vascular involvement

pathergy test