Hughes-Stovin syndrome (HSS) is a rare autoimmune disease, considered a variant of Behçet syndrome, characterized by the appearance of pulmonary artery aneurysms and peripheral deep vein thrombosis, not a contagious disease. .
Usually appears after the third decade of life.
It is often fatal due to the rupture of pulmonary artery aneurysms (Pulmonary Artery Aneurysm, PAA).
HistoryThe syndrome was described by John Patterson Hughes and Peter George Stovin in 1959, after attending two cases of hemoptysis for rupture of pulmonary artery aneurysms and peripheral venous thrombosis. symptom Treatment
The treatment is aimed at reducing inflammation of the aneurysms and suppressing the patient's immune system, since Hughes-Stovin has no cure.
It is treated with beta-blockers; and immunosuppressants and corticosteroids: prednisolone, azathioprine.
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