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?

Is there a seasonal variation in the incidence of cardiovascular (CV) events or venous thromboembolism (VTE)?

Seasonal variation, primarily characterized by a winter peak, has been reported for acute CV events, such as acute myocardial infarction (AMI) and sudden death, aortic rupture or dissection, and ischemic or hemorrhagic stroke, and VTE (1). A meta-analysis involving patients with VTE, primarily with a diagnosis of pulmonary embolism, revealed a 20% absolute increase in the incidence of VTE during January (1).  

Potential physiological mechanisms for these observations include increased sympathetic activity, decreased loss of fluids and sodium, increase in LDL cholesterol, increase in serum fibrinogen levels and other coagulation markers, and C-reactive protein, and lower vitamin D levels due to shorter daylight hours during winter months (1,2). At least in the case of AMI in the U.S., the higher incidence in winter is not affected by climate (2).  Respiratory virus infections as a cause of acute inflammation leading to  CV or VTE events is an intriguing hypothesis (3).

 

References

  1. Dentali F, Ageno W, Rancan E, et al. Seasonal and monthly variability in the incidence of venous thromboembolism. A systematic review and a meta-analysis of the literature. Thromb Haemost 2011;106:439-447.
  2. Spencer FA, Goldberg RJ, Becker RC, et al. Seasonal distribution of acute myocardial infarction in the Second National Registry of Myocardial Infarction. J Am Coll Cardiol 1998;31:1226-33.
  3. 3. Woodhouse PR, Khaw KT, Plummer M, et al. Seasonal variations of plasma fibrinogen and factor VII activity in the elderly: winter infections and death from cardiovascular disease. Lancet 1994;343:435-39.

 

 

 

 

Is there a seasonal variation in the incidence of cardiovascular (CV) events or venous thromboembolism (VTE)?