CHEST Guidelines-Recurrent-Pulmonary-Aneurysms

Recurrent-Pulmonary-Aneurysms_chest (1)

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The report describes a case of a 34-year-old South African man with recurrent life-threatening hemoptysis due to multiple pulmonary aneurysms, which led to a diagnosis of Hughes-Stovin Syndrome and later, Behçet disease. Initially presenting with eye problems suggestive of panuveitis, he was subsequently readmitted with massive hemoptysis, cough, fever, and significant vascular anomalies revealed through CT angiography. Attempts to treat the aneurysms using coil embolization and a right lower lobectomy were only partially successful.<br /><br />Further complications included extensive lower body venous thrombosis extending to the inferior vena cava, making standard anticoagulation risky. After interventions for recurrent hemoptysis and failed embolizations, prednisone and azathioprine treatments showed improvement. The diagnosis of Behçet disease was solidified through the patient’s recurrent oral ulcers, positive pathergy testing, and the ophthalmological history.<br /><br />Behçet disease is characterized by recurrent inflammation affecting the mucous membranes and various organs, and the Hughes-Stovin syndrome observed in the patient is discussed as possibly a sub-type rather than a separate entity within Behçet’s spectrum due to overlapping symptoms like severe bleeding and thrombosis.<br /><br />Despite challenges in managing the patient’s dual symptoms, the case demonstrated effective control of the pulmonary aneurysms with immunosuppressive therapy. The patient responded well and demonstrated no further hemoptysis following treatment, contributing to the broader discourse on these interconnected, yet complex diseases. This case underscores the importance of considering both Behçet disease and Hughes-Stovin syndrome in patients with unexplained vascular involvement and associative symptoms.

Keywords

Hughes-Stovin Syndrome

Behçet disease

pulmonary aneurysms

hemoptysis

panuveitis

vascular anomalies

coil embolization

venous thrombosis

immunosuppressive therapy

pathergy testing