Epidemiology

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

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Physiology

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

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Diagnosis

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

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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

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Treatment

• xxx

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References

• xxx