ABSTRACT
El síndrome de Hughes Stovin es una vasculitis de pequeños y grandes vasos, más frecuente en hombres jóvenes. Se manifiesta con trombosis y aneurismas. Inicia con tromboflebitis que evoluciona a aneurismas con eventual desenlace fatal por ruptura de los mismos. Algunos autores consideran el síndrome de Hughes Stovin como una variante de la enfermedad de Behçet. El diagnóstico es clínico y el tratamiento de primera línea son los corticoides y la ciclofosfamida. Frente a un caso de trombosis, la anticoagulación es controversial por el riesgo de sangrado. Presentamos el caso de un varón joven con cuadro clínico compatible en el cual se descartaron otras patologías con requerimiento de tratamiento quirúrgico por mala evolución clínica.
Hughes Stovin syndrome is characterized by small and big vessels vasculitis, more commonly presented in young men. Initially it manifests with thrombophlebitis and aneurysms which can cause fatal bleed due to rupture. Some authors consider Hughes Stovin syndrome as a variant of Behcet's disease. Diagnosis is made by clinical findings and the first line treatment are corticosteroids and cyclophosphamide. Anticoagulation is controversial due to the increased risk of hemorrhage.
Subject(s)
MaleABSTRACT
Hughes-Stovin syndrome is a rare disorder of unknown etiology. Although the association between multiple pulmonary artery aneurysms and venous thrombosis of the lower limbs was reported by Beattie and Hall in 1911, it was not until 1962 that the eponym “Hughes-Stovin syndrome” was formally introduced in the medical literature. We describe 2 patients with Hughes-Stovin syndrome who presented with pulmonary artery aneurysm, thrombophlebitis, hemoptysis, and oral ulcers, review the manifestations of the disease, and compare its similarities with and differences from Behçet disease.
Subject(s)
Humans , Aneurysm , Behcet Syndrome , Eponyms , Hemoptysis , Lower Extremity , Oral Ulcer , Pulmonary Artery , Thrombophlebitis , Thrombosis , Venous ThrombosisABSTRACT
El síndrome de Hughes-Stovin (SHS) es una enfermedad rara, caracterizada por la presencia de tromboflebitis y aneurismas de las arterias pulmonares y/o bronquiales. Si bien la etiología y patogénesis del SHS se desconoce, se han identificado asociaciones con ciertas infecciones y angiodisplasia. Pertenece al grupo de las vasculitis generalizadas, con un mecanismo patológico muy parecido al de la enfermedad de Behçet (EB), incluso considerándose como una variante de esta última. Las manifestaciones clínicas incluyen tos, disnea, fiebre, toracodinia y hemoptisis. Es fundamental realizar un diagnóstico y tratamiento de manera temprana debido a que estos pacientes presentan un alto riesgo de ruptura de los aneurismas de arteria pulmonar. El tratamiento se basa en el uso de agentes inmunosupresores.
The Hughes-Stovin Syndrome (HSS) is a very rare clinical disorder characterized by thrombophlebitis and multiple pulmonary and/or bronchial aneurysms. The exact etiology and pathogenesis of HSS is unknown; possible causes include infections and angiodysplasia. HSS has also been considered as a variant of Behcet's disease (BD). Patients with HSS usually present with cough, dyspnea, fever, chest pain and haemoptysis. Early diagnosis and treatment are important, because patients are at a high risk for devastating pulmonary artery aneurysm rupture. Treatment usually involves immunosuppressive agents.
Subject(s)
Thrombophlebitis , Angiodysplasia , AneurysmABSTRACT
Pulmonary and/or bronchial aneurysm with systemic thrombophlebitis can be seen in the case of both Behcet's disease and Hughes-Stovin syndrome. These two diseases may be indistinguishable since the clinical, radiological, and histopathological findings are similar in both cases. In particular, from the perspective of pulmonary involvement, hemoptysis is the leading cause of death in both conditions and is followed by aneurysmal rupture. Here, we report a case in which surgical resection was performed for a patient presenting recurrent hemoptysis as an atypical presentation of Behcet's disease.
Subject(s)
Humans , Aneurysm , Behcet Syndrome , Cause of Death , Hemoptysis , Pulmonary Artery , Rupture , ThrombophlebitisABSTRACT
We report a 25yearold mole admitted to a critical care unit for fever, lung opacities and acute respiratory failure. A chest angio-CAT sean showed multiple pulmonary artery aneurysms. A deep venous thrombosis of both lower limbs was also documented. A Hughes-Stovin syndrome was postulated. An inferior vena cava filter was placed. The patient received antimicrobial therapy subsiding fever and respiratory failure. Subsequently, he was treated with intravenous and oral steroids and one dose of cyclophosphamide. The patient was discharged in good conditions fifteen days after admission.
Subject(s)
Adult , Humans , Aneurysm/diagnosis , Pulmonary Artery , Venous Thrombosis/diagnosis , Aneurysm/therapy , Chile , Syndrome , Tomography, X-Ray Computed , Venous Thrombosis/therapyABSTRACT
A síndrome de Hughes-Stovin é uma condição rara, de causa desconhecida, caracterizada pela associação de múltiplos aneurismas de artéria pulmonar e trombose venosa profunda. Alguns autores consideram tal entidade como uma forma incompleta de apresentação da doença de Behçet, devido à semelhança entre os achados radiológicos e anatomopatológicos do comprometimento pulmonar. Os autores relatam um caso de síndrome de Hughes-Stovin cujo primeiro evento trombótico venoso antecedeu em cinco anos o aparecimento dos aneurismas pulmonares.
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.
Subject(s)
Humans , Male , Adult , Aneurysm , Pulmonary Embolism/diagnosis , Pulmonary Artery , Pulmonary Veins , ThrombosisABSTRACT
Hughes-Stovin Syndrome is an exceedingly rare combination of distal pulmonary arterial aneurysm and deep vein thrombosis, mostly found in young patients. There are striking similarities between the vascular manifestation of Behcet's disease and Hughes-Stovin Syndrome. It has been suggested that they may have a similar pathogenesis. Most patients died of massive hemoptysis due to rupture of aneurysm. Recently we have experienced the first case of Hughes-Stovin Syndrome in Korea. A 37 year old male patient was admitted because of recurrent hemoptysis and intermittent fever. He had a history of recurrent aphthous ulcers and erythema nodosum-like skin rash, But no other findings of Behcet's disease was found. Angiography showed multiple pulmonary arterial aneurysm and deep vein thrombosis in Right lower extremity. Histologic examination of specimens of open lung biopsy revealed leukocyto- clastic angiitis. Pulmonary arterial aneurysms were successfully treated by coil embolization and he is in good condition with corticosteroid and cyclophosphamide therapy.