It occurs predominantly between the 2nd to 4th decades. There is a recognized male predilection.
Typically affects pulmonary, and frequently bronchial, arteries and large systemic veins.
Typically disease is clinically characterized 3 phases 5:
- stage I: thrombophlebitis
- stage II: formation and enlargement of pulmonary aneurysms
- stage III: aneurysmal rupture
Exact features will depend on the stage. May show:
- systemic thrombi in the vena cava, cerebral sinuses, or limb veins
- pulmonary arterial occlusions due to emboli or thrombi
- one or more segmental pulmonary arterial aneurysms
- bronchial arterial aneurysms.
- massive pulmonary hemorrhage (stage III): from a rupture of a pulmonary arterial aneurysm; often a terminal event 4.
History and etymology
It is named after J P Hughes and P G Stovin who first described the condition in 1959 6.
General imaging differential considerations include:
- 1. Marten K, Schnyder P, Schirg E et-al. Pattern-based differential diagnosis in pulmonary vasculitis using volumetric CT. AJR Am J Roentgenol. 2005;184 (3): 720-33. AJR Am J Roentgenol (full text) - Pubmed citation
- 2. Khalil A, Parrot A, Fartoukh M et-al. Images in cardiovascular medicine. Large pulmonary artery aneurysm rupture in Hughes-Stovin syndrome: multidetector computed tomography pattern and endovascular treatment. Circulation. 2006;114 (10): e380-1. doi:10.1161/CIRCULATIONAHA.106.614636 - Pubmed citation
- 3. Ammann ME, Karnel F, Olbert F et-al. Radiologic findings in the diagnosis of Hughes-Stovin syndrome. AJR Am J Roentgenol. 1991;157 (6): 1353-4. - Pubmed citation
- 4. Kindermann M, Wilkens H, Hartmann W et-al. Images in cardiovascular medicine. Hughes-Stovin syndrome. Circulation. 2003;108 (23): e156. doi:10.1161/01.CIR.0000106680.35316.16 - Pubmed citation
- 5. Ketchum ES, Zamanian RT, Fleischmann D. CT angiography of pulmonary artery aneurysms in Hughes-Stovin syndrome. AJR Am J Roentgenol. 2005;185 (2): 330-2. AJR Am J Roentgenol (full text) - Pubmed citation
- 6. Hughes JP, Stovin PG. Segmental pulmonary artery aneurysms with peripheral venous thrombosis. Br J Dis Chest. 1959;53 (1): 19-27. - Pubmed citation