RESUMO
The Hughes-Stovin syndrome is a rare entity characterized by deep vein thrombosis and pulmonary artery aneurysms of unknown etiology and pathogenesis. Some authors considered a variant of Behcet's disease. Its natural course is usually fatal. The symptoms are cough, dyspnea, hemoptysis, chest pain and fever. The treatment goes from steroids and cytotoxic agents to surgery. We present the case of a 41 year old man who shows dyspnea, hemoptysis, and chest pain leading to the diagnosis of deep venous thrombosis of the right leg, lung thromboembolism and pulmonary artery aneurysms. He was treated with high-dose corticosteroids and 6 cyclophosphamide pulses of 1 gram each per 6 months with complete regression of aneurysms and symptomatology.
Assuntos
Aneurisma Roto/diagnóstico , Artéria Pulmonar , Embolia Pulmonar/diagnóstico , Trombose Venosa/diagnóstico , Adulto , Síndrome de Behçet/diagnóstico , Hemoptise/etiologia , Humanos , Masculino , SíndromeRESUMO
El síndrome de Hughes-Stovin es una entidad infrecuente caracterizada por trombosis venosa profunda y aneurismas de la arteria pulmonar, siendo su etiología y patogenia desconocida. Algunos autores la consideran una variante de la enfermedad de Behcet. Su curso natural es generalmente fatal. Se presenta con tos, disnea, hemoptisis, dolor torácico y fiebre. El tratamiento es con esteroides y agentes citotóxicos hasta la cirugía. Presentamos el caso de un hombre de 41 años que consultó por disnea, hemoptisis y dolor torácico, llegándose al diagnóstico de trombosis venosa profunda de miembro inferior derecho, trombo-embolismo de pulmón y aneurismas de arterias pulmonares. Recibió tratamiento con corticoides en altas dosis y 6 pulsos de ciclofosfamida de 1 gramo durante 6 meses, con regresión completa de los aneurismas y de la sintomatología.(AU)
The Hughes-Stovin syndrome is a rare entity characterized by deep vein thrombosis and pulmonary artery aneurysms of unknown etiology and pathogenesis. Some authors considered a variant of Behcets disease. Its natural course is usually fatal. The symptoms are cough, dyspnea, hemoptysis, chest pain and fever. The treatment goes from steroids and cytotoxic agents to surgery. We present the case of a 41 year old man who shows dyspnea, hemoptysis, and chest pain leading to the diagnosis of deep venous thrombosis of the right leg, lung thromboembolism and pulmonary artery aneurysms. He was treated with high-dose corticosteroids and 6 cyclophosphamide pulses of 1 gram each per 6 months with complete regression of aneurysms and symptomatology.(AU)
RESUMO
El síndrome de Hughes-Stovin es una entidad infrecuente caracterizada por trombosis venosa profunda y aneurismas de la arteria pulmonar, siendo su etiología y patogenia desconocida. Algunos autores la consideran una variante de la enfermedad de Behcet. Su curso natural es generalmente fatal. Se presenta con tos, disnea, hemoptisis, dolor torácico y fiebre. El tratamiento es con esteroides y agentes citotóxicos hasta la cirugía. Presentamos el caso de un hombre de 41 años que consultó por disnea, hemoptisis y dolor torácico, llegándose al diagnóstico de trombosis venosa profunda de miembro inferior derecho, trombo-embolismo de pulmón y aneurismas de arterias pulmonares. Recibió tratamiento con corticoides en altas dosis y 6 pulsos de ciclofosfamida de 1 gramo durante 6 meses, con regresión completa de los aneurismas y de la sintomatología.
The Hughes-Stovin syndrome is a rare entity characterized by deep vein thrombosis and pulmonary artery aneurysms of unknown etiology and pathogenesis. Some authors considered a variant of Behcet's disease. Its natural course is usually fatal. The symptoms are cough, dyspnea, hemoptysis, chest pain and fever. The treatment goes from steroids and cytotoxic agents to surgery. We present the case of a 41 year old man who shows dyspnea, hemoptysis, and chest pain leading to the diagnosis of deep venous thrombosis of the right leg, lung thromboembolism and pulmonary artery aneurysms. He was treated with high-dose corticosteroids and 6 cyclophosphamide pulses of 1 gram each per 6 months with complete regression of aneurysms and symptomatology.
Assuntos
Humanos , Masculino , Adulto , Artéria Pulmonar , Embolia Pulmonar/diagnóstico , Aneurisma Roto/diagnóstico , Trombose Venosa/diagnóstico , Síndrome , Síndrome de Behçet/diagnóstico , Hemoptise/etiologiaRESUMO
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.