What complication of Behçet’s syndrome carries the highest mortality?

Behçet’s syndrome may cause life-threatening hemoptysis due to pulmonary artery aneurysms.1 In a cohort of 387  patients with such syndrome followed for over 20 years, massive hemoptysis was the leading cause of death, found most commonly early in the course of the disease among young men.2 Conversely, the one-year mortality of pulmonary artery aneurysm in Behçet’s may be greater than 50%.1 Behçet’s syndrome is the only vasculitic disease with a proclivity for large pulmonary vessels, while its less frequent pulmonary manifestations, such as fibrosis and thrombosis, overlap with other small vessel vasculitides.3 Beware that the initial presentation of pulmonary aneurysm rupture may be confused with that of pulmonary embolism, with potential for fatal complications from anticoagulation.1 CT angiogram should help in distinguishing the two conditions.

 

References 

  1. Uzun O, Akpolat T, Erkan L. Pulmonary vasculitis in behcet disease: a cumulative analysis. Chest. 2005;127(6):2243-2253.
  2. Kural-Seyahi E, Fresko I, Seyahi N, et al. The long-term mortality and morbidity of Behçet syndrome: a 2-decade outcome survey of 387 patients followed at a dedicated center. Medicine (Baltimore). 2003;82(1):60-76.
  3. Hamuryudan V, Er T, Seyahi E, et al. Pulmonary artery aneurysms in Behçet syndrome. Am J Med. 2004;117(11):867-870.

 

Contributed by Sam Slavin, Harvard Medical Student

What complication of Behçet’s syndrome carries the highest mortality?

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