Hughes-Stovin syndrome is a very rare disease with fewer than 40 cases reported in the literature. The disease is thought to be a variant of Behçet's disease that can manifest with massive haemoptysis due to ruptured pulmonary arterial aneurysms in the setting of deep venous thrombosis. This association might impose a therapeutic dilemma when anticoagulation has to be balanced with the excessive risk of bleeding, especially if the patient refuses any blood replacement, and is not a surgical candidate. We present a case of a 41-year-old Jehovah's Witness patient with Hughes-Stovin syndrome that presented to the emergency room with haemoptysis due to multiple ruptured pulmonary artery aneurysms. Her work-up showed concomitant cardio-venous thromboembolism and pulmonary infarction. She was successfully managed via a bloodless single-wall venous puncture, allowing endovascular treatment of bilateral pulmonary aneurysms with subsequent caval filtration. The course was uneventful, and the patient was discharged under immunosuppressant.
- Hughes-stovin syndrome
ASJC Scopus subject areas
- Cardiology and Cardiovascular Medicine
- Pulmonary and Respiratory Medicine