Hughes-Stovin Syndrome

Epidemiology

  • First reported in 1959
  • Probably represents a variant of Behcet s syndrome

Physiology

  • Vasculitis: lypmhocyte and plasma cell infiltrate in walls of elastic pulmonary arteries

Diagnosis

  • PFT’s: decreased DLCO/ increased Vd/Vt ratio
  • ABG: elevated A-a gradient
  • OLB: lymphocyte and plasma cell infiltrate in elestic PA

Clinical

  • Pulmonary Manifestations
    • Segmental Pulmonary Artery Aneurysms
    • Massive Fatal Hemoptysis: due to rupture of segmental pulmonary artery aneurysms
  • Other Manifestations
    • Recurrent Venous Thrombosis:
    • Intracranial Hypertension: due to thrombosis of intracranial and extra cranial vessels

Treatment

  • xxx

References

  • xxx