Clinical Pearls & Morning Reports
Published April 11, 2018
A pulmonary artery aneurysm is a focal dilatation of all three layers of the vessel wall, whereas a pseudoaneurysm does not involve all the layers. Pulmonary artery aneurysms are treated aggressively because of the morbidity and mortality associated with rupture. Read the latest NEJM Case Records of the Massachusetts General Hospital here.
Q: What inflammatory diseases are associated with pulmonary artery aneurysms?
A: The inflammatory diseases that are associated with pulmonary artery aneurysms are rare and include Takayasu’s arteritis, Behçet’s disease, and the Hughes–Stovin syndrome. The pulmonary artery aneurysms that develop in patients with Takayasu’s arteritis are usually poststenotic dilatations.
Q: How are pulmonary artery aneurysms associated with Behçet’s disease managed?
A: Because there is considerable evidence that formation of both thrombosis and aneurysm in Behçet’s disease is caused by inflammation rather than a hypercoagulable state, immunosuppression is the cornerstone of treatment. The role of anticoagulation in Behçet’s disease is uncertain.
A: Although pulmonary artery aneurysms are rare, several well-described conditions are associated with their development, including trauma, infection, congenital heart disease, lung cancer, pulmonary hypertension, and systemic inflammatory diseases. Infections that may cause pulmonary artery aneurysms include syphilis and tuberculosis. Endocarditis with septic emboli to the pulmonary arterial system can cause pulmonary artery aneurysms. Pulmonary artery aneurysms can be associated with congenital heart disease, such as patent ductus arteriosus. On rare occasions, pulmonary artery aneurysms have been associated with lung cancer. Pulmonary artery aneurysms that are associated with pulmonary hypertension are usually observed in longstanding, severe disease.
A: Behçet’s disease and the Hughes–Stovin syndrome are the two vasculitides that are most commonly associated with the development of pulmonary artery aneurysms. These two diagnoses share many characteristics, and it has been suggested that the Hughes– Stovin syndrome is a partially manifested form of Behçet’s disease; indeed, the Hughes–Stovin syndrome has been referred to as “a cardiovascular manifestation of Behçet’s disease” and “incomplete Behçet’s.” The Hughes–Stovin syndrome is an extremely rare disease, with fewer than 40 cases reported in the literature. Aneurysms in the Hughes–Stovin syndrome can be single or multiple and can be unilateral or bilateral; they usually involve the pulmonary and bronchial arteries but can occur anywhere in the systemic circulation. Three phases are described in the Hughes–Stovin syndrome: an initial phase characterized by venous thrombophlebitis, a second phase characterized by formation of large pulmonary and bronchial aneurysms, and a third phase characterized by aneurysmal rupture that leads to massive hemoptysis and death.