Hughes-Stovin syndrome

Hughes-Stovin syndrome (HSS) is a vasculitis that predominantly affects large vessels. The disease bears some resemblance to Behcet disease.

It predominantly occurs predominantly between the 2nd to 4th decades. There is a recognised male predilection. 

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 

Exact features will depend on the stage. May show:

  • massive pulmonary haemorrhage (stage III): from a rupture of a pulmonary arterial aneurysm; often a terminal event 4.

It is named after J P Hughes and P G Stovin who first described the condition in 1959 6.

General imaging differential considerations include:

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Article Information

rID: 12482
System: Vascular
Synonyms or Alternate Spellings:
  • Hughes Stovin syndrome
  • Hughes Stovin syndrome (HSS)
  • HSS

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