Hughes-Stovin syndrome
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Hughes-Stovin syndrome (HSS) is a vasculitis that predominantly affects large vessels. The disease bears some resemblance to BehcetBehçet disease.
Epidemiology
It occurs predominantly between the 2nd to 4th decades. There is a recognised male predilection.
Pathology
Distribution
Typically affects pulmonary, and frequently bronchial, arteries and large systemic veins.
Progression
Typically disease is clinically characterised 3 phases 5:
- stage I: thrombophlebitis
- stage II: formation and enlargement of pulmonary aneurysms
- stage III: aneurysmal rupture
Radiographic features
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.
Complications
- massive pulmonary haemorrhage (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.
Differential diagnosis
General imaging differential considerations include:
-<p><strong>Hughes-Stovin syndrome (HSS)</strong> is a <a href="/articles/vasculitis">vasculitis</a> that predominantly affects large vessels. The disease bears some resemblance to <a href="/articles/behcet-disease-2">Behcet disease</a>.</p><h4>Epidemiology</h4><p>It occurs predominantly between the 2<sup>nd</sup> to 4<sup>th</sup> decades. There is a recognised male predilection. </p><h4>Pathology</h4><h5>Distribution </h5><p>Typically affects pulmonary, and frequently bronchial, arteries and large systemic veins. </p><h5>Progression</h5><p>Typically disease is clinically characterised 3 phases <sup>5</sup>:</p><ul>- +<p><strong>Hughes-Stovin syndrome (HSS)</strong> is a <a href="/articles/vasculitis">vasculitis</a> that predominantly affects large vessels. The disease bears some resemblance to <a href="/articles/behcet-disease-2">Behçet disease</a>.</p><h4>Epidemiology</h4><p>It occurs predominantly between the 2<sup>nd</sup> to 4<sup>th</sup> decades. There is a recognised male predilection. </p><h4>Pathology</h4><h5>Distribution </h5><p>Typically affects pulmonary, and frequently bronchial, arteries and large systemic veins. </p><h5>Progression</h5><p>Typically disease is clinically characterised 3 phases <sup>5</sup>:</p><ul>
-</ul><h4>Complications</h4><ul><li>massive <a href="/articles/pulmonary-haemorrhage">pulmonary haemorrhage</a> (stage III): from a rupture of a pulmonary arterial aneurysm; often a terminal event <sup>4</sup>.</li></ul><h4>History and etymology</h4><p>It is named after <strong>J P Hughes</strong> and <strong>P G Stovin</strong> who first described the condition in 1959 <sup>6</sup>.</p><h4>Differential diagnosis</h4><p>General imaging differential considerations include:</p><ul><li><a href="/articles/behcet-disease-2">Behcet disease</a></li></ul>- +</ul><h4>Complications</h4><ul><li>massive <a href="/articles/pulmonary-haemorrhage">pulmonary haemorrhage</a> (stage III): from a rupture of a pulmonary arterial aneurysm; often a terminal event <sup>4</sup>.</li></ul><h4>History and etymology</h4><p>It is named after <strong>J P Hughes</strong> and <strong>P G Stovin</strong> who first described the condition in 1959 <sup>6</sup>.</p><h4>Differential diagnosis</h4><p>General imaging differential considerations include:</p><ul><li><a href="/articles/behcet-disease-2">Behçet disease</a></li></ul>
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