Síndrome de Hughes-Stovin

Abstract

Hughes-Stovin syndrome is a rare disorder of unknown etiology characterized by the association of multiple pulmonary artery aneurysms and deep venous thrombosis. Some authors consider this entity an incomplete form of Behcet's disease due to the similarities between the radiologic and anatomopathological findings of pulmonary involvement. The authors report a case of Hughes-Stovin syndrome whose first venous thrombotic event preceded the development of pulmonary aneurysms by five years.

Life threatening hemoptysis from Hughes Stovin syndrome: Is it that rare?

“…Hughes-Stovin syndrome clinically passes into three phases: a first phase involving symptoms of thrombophlebitis, a second phase involving formation of pulmonary artery aneurysms, and a third phase of aneurysmal rupture that results in massive hemoptysis that is usually fatal [3] . Accordingly, we do think that this condition is not very uncommon.…”

Hughes-Stovin syndrome: an unusual cause of pulmonary artery aneurysms

“…Idiopathic and vascular diseases of the thorax have been the subject of recent publications in the radiology literature of Brazil ( 1 - 6 ) . Hughes-Stovin syndrome is a rare condition, characterized by the combination of multiple pulmonary artery aneurysms and peripheral venous thrombosis, that mainly affects males (80-90% of cases) between the second and fourth decades of life ( 7 - 11 ) . Although the lesions often affect arteries and veins simultaneously (in 68% of cases), isolated arterial or venous impairments are reported at frequencies of 25% and 7%, respectively ( 9 , 11 ) .…”

“…In its typical presentation, Hughes-Stovin syndrome occurs in three stages ( 7 - 9 , 11 ) : in the first stage, there are signs and symptoms of thrombophlebitis; the second stage includes the formation and expansion of pulmonary artery aneurysms; and the third stage is characterized by aneurysmal rupture with massive hemoptysis, progressing to death. The formation of pulmonary aneurysms has been attributed to the weakening of the vessel walls by an inflammatory process.…”

“…Some authors consider Hughes-Stovin syndrome an incomplete form of Behcet's disease, due to the similarity between the two in terms of the clinical, radiological, and pathological findings ( 7 - 11 ) . Therefore, Behcet's disease, which typically affects young males, is the main differential diagnosis ( 11 ) .…”

“…There is no standard treatment for Hughes-Stovin syndrome, the most widely used treatment option being immunosuppression therapy involving a combination of glucocorticoids and cyclophosphamide, which has the potential to stabilize aneurysms or even promote regression in some cases ( 11 ) . The use of anticoagulants is controversial because of the risk of fatal hemoptysis, being allowed only in selected cases and provided jointly administered with immunosuppression therapy ( 7 - 11 ) . Other possible treatments include surgical resection and arterial embolization, which are used in most cases in which there is massive hemoptysis ( 11 ) .…”