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Hughes-Stovin syndrome
Epidemiology:
- extremely rare (< 40 cases reported)
Clinical manifestations:
phase 1
- thrombophlebitis
- chronic venous thromboembolism
phase 2
- vascular aneurysm
- pulmonary artery aneurysm
- bronchial artery aneurysm
- can be multiple or anywhere in the systemic circulation
phase 3
- aneurysm rupture
- hemoptysis
- massive hemorrhage & death
Laboratory:
- no diagnostic tests
Radiology:
- pulmonary CT angiography
Differential diagnosis:
- Behcet syndrome
Management:
- immunosuppresion
- glucocorticoids, cyclophosphamide & infliximab*
* inflimi
Related
Behcet syndrome
General
vasculitis
syndrome
References
- Farber HW, McDermott S, Witkin AS et al
Case 11-2018: A 48-Year-Old Woman with Recurrent Venous
Thromboembolism and Pulmonary Artery Aneurysm.
N Engl J Med 2018; 378:1430-1438. April 12, 2018
PMID: 29641962
http://www.nejm.org/doi/full/10.1056/NEJMcpc1800323