Hughes stovin syndrome is a rare condition characterized by peripheral deep venous thrombosis accompanied by single or multiple pulmonary arterial aneurysms. Jan 29, 2009 hughesstovin syndrome is a rare entity. He also appeared to have a longstanding dural venous sinus thrombosis. Successful treatment of refractory hughes stovin syndrome with infliximab successful treatment of refractory hughes stovin syndrome with infliximab. If you have a great topic or idea, you can propose a special issue and you will have the opportunity to be the lead guest editor of the special issue. Review open access hughes stovin syndrome umair khalid and taimur saleem abstract hughes stovin syndrome hss is a very rare clinical disorder characterized by thrombophlebitis and multiple pulmonary andor bronchial aneurysms. Hughesstovin syndrome hss is a vasculitis that predominantly affects large vessels. Hughesstovin syndrome hss is a very rare clinical disorder characterized by thrombophlebitis and multiple pulmonary andor bronchial aneurysms.
The underlying pathophysiology and treatment is not clearly understood owing to the lack of quality case studies. It has been described in literature less than 40 times. Arterial blood culture repeatedly examined had been negative. A 25yearold woman was found to have hughes stovin syndrome, previously reported only in males.
Hughes stovin syndrome information including symptoms, causes, diseases, symptoms, treatments, and other medical and health issues. Hughes stovin syndrome is a very rare pathology of unknown etiology with a lethal potential. The treatment of hughesstovin syndrome is similar to behcets diseasesteroids alone or in combination with immunosuppressants. Pdf hughesstovin syndrome is a rare disorder of unknown etiology characterized by the association of multiple pulmonary artery. Hughesstovin syndrome hss is a rare disorder, first described in 1959 by j p. Firstly, various antibiotic regimens that have been tried in the treatment of hss have proven ineffective. It is characterized by the association of pulmonary artery aneurysms and peripheral venous thrombosis. Special issue special issue is an effective way for researchers to focus on a hot topic for an indepth study. Hughesstovin syndrome is a rare autoimmune disorder of unknown cause that is characterized by the combination of multiple pulmonary artery aneurysms and deep vein thrombosis. The limited number of cases has precluded controlled studies of the management of pulmonary artery aneurysms, which usually cause massive hemoptysis leading to death. Massive pulmonary hemorrhage caused by rupture of an aneurysm is a frequent terminal event. The diagnosis of hughesstovin syndrome was made and the patient was treated with steroids and cyclophosphamide. Background hughes stovin syndrome is a rare entity. Hughesstovin syndrome hss is a lifethreatening disorder, believed to be a.
The clinical presentation may include cough or hemoptysis and venous thromboses either in the extremities, the dural sinuses, the vena cavae, or the right atrium. Ct angiography of pulmonary artery aneurysms in hughes. Patients affected by this syndrome are often young adult men in the second to fourth decade of life. Less than 40 cases have ever been reported in the english medical literature. Hughesstovin syndrome article about hughesstovin syndrome. Large pulmonary artery aneurysm rupture in hughesstovin syndrome. Abstract hughes stovin syndrome hss is a very rare clinical disorder characterized by thrombophlebitis and multiple pulmonary andor bronchial aneurysms. This rare entity may be suspected when a relatively young patient presents with evidence of thromboembolic pulmonary disease, with one or more enlarging pulmonary masses associated with evidence of systemic venous thrombosis vena cava, peripheral veins, or dural sinuses. An extraordinary cause in the etiology of recurrent. The clinical condition of the patient improved at the 4month followup. Hughesstovin syndrome definition of hughesstovin syndrome.
Stovin syndrome and was the first female case of typical hughes. Hughesstovin syndrome with pulmonary angiitis and focal. A patient is reported with pulmonary arterial occlusions and aneurysms and recurrent haemoptysis. Less than 40 published cases of hss have been described in. It is characterized by the association of multiple pulmonary artery aneurysms paas and peripheral venous thrombosis. Hughes stovin syndrome is a very rare clinical entity characterized by pulmonary artery aneurysms and deep vein thrombosis dvt.
Hughesstovin syndrome is characterized by multiple pulmonary and or bronchial artery aneurysms and thrombophlebitis. Hughesstovin syndrome is a rare disorder of unknown etiology characterized by the association of multiple pulmonary artery aneurysms and deep venous thrombosis. Our report underlines the importance of tnf blocker treatment in hughes stovin syndrome, suggesting its use as long. Review of the literature revealed 9 typical cases of this syndrome and 5 atypical cases who had solitary intrapulmonary. Objectives to illustrate the use of infliximab in three cases. Successful closure of pulmonary artery aneurysm in a patient. Stovin, 1 with fewer than 40 cases reported worldwide2, 3 and most commonly affecting young men aged 1240 years.
This is the first report of a new endovascular treatment of a single. The hughes stovin syndrome consists of deep venous thrombosis often involving the caval vein accompanied by single or multiple pulmonary arterial aneurysms. Hughes stovin syndrome pdf hughes stovin syndrome is a rare autoimmune disorder of unknown cause that is characterized by the combination of multiple pulmonary artery aneurysms and. Aps provokes blood clots in both arteries and veins as well as pregnancyrelated complications such as miscarriage, stillbirth, preterm delivery, and severe preeclampsia. We report the case of an 18 years old, greek male patient with hughesstovin syndrome, who initially presented with deep vein thrombosis. The disease bears some resemblance to behcet disease.
Ct angiography of pulmonary artery aneurysms in hughesstovin. Massive hemoptysis and deep venous thrombosis presenting in a. Hughesstovin syndrome without venous involvement unusual. The aetiology of hughes stovin syndrome is still unknown and the natural course of the illness is usually fatal. Apr, 2011 hughes stovin syndrome hss is a very rare clinical disorder characterized by thrombophlebitis and multiple pulmonary andor bronchial aneurysms. Although most are asymptomatic at diagnosis, fatal aneurysm rupture occurs in onethird and dissection in onefifth.
Hughes stovin syndrome vitor alves cruz1, yadine a. Hughes stovin syndrome is characterized by multiple pulmonary and or bronchial artery aneurysms and thrombophlebitis. Antiphospholipid syndrome, or antiphospholipid antibody syndrome aps or apls, is an autoimmune, hypercoagulable state caused by antiphospholipid antibodies. Hughesstovin syndrome is an extremely rare autoimmune clinical disorder that is character ized by deep vein thrombosis with multiple pulmonary andor bronchial arterial aneurysms. He gave a history of recurrent arthropathy and febrile illnesses. The hughesstovin syndrome consists of deep venous thrombosis often involving the caval vein. The exact etiology and pathogenesis of hss is unknown. Life threatening hemoptysis from hughes stovin syndrome.
Hughesstovin syndrome orphanet journal of rare diseases. Background hughes stovin syndrome hss is described as a very rare vascular manifestation of behcets disease in medical literature. The patient received treatment in the form of intravenous pulse methyl. Hughes stovin syndrome umair khalid and taimur saleem abstract hughes stovin syndrome hss is a very rare clinical disorder characterized by thrombophlebitis and multiple pulmonary andor bronchial aneurysms. Turkish venercologist halushi behcet described the constellation of hypopyon, iritis and orogential ulcers in 4. Hughesstovin syndrome is a lifethreatening disorder of unknown etiology. The exact etiology and pathogenesis of hss is unknown, possible causes include infections and angiodysplasia. Pulmonary medium vessel vasculitis in an 11 year old boy. Also, the thrombin in the lower extremities is tightly adherent to the inflamed veins in bd and hss patients 121937. Hss is a very rare clinical disorder and around 4060 published cases of hss have been described in medical literature so far. The etiopathogenesis of hughes stovin syndrome remains unexplained. It is named after the two british physicians, john patterson hughes and peter george ingle stovin, who first described it in 1959. The typical clinical presentation of hughesstovin syndrome consists of three phases. Hughesstovin syndrome hss is a very rare clinical disorder characterized by.
Hughesstovin syndrome hss is a very rare condition characterized by pulmonary aneurysms and peripheral thrombophlebitis of lower limbs. Patients with hughesstovin syndrome can present with massive hemoptysis from. The typical clinical presentation of hughes stovin syndrome consists of three phases. Here we report the case of a 53yearold man, admitted to sultan qaboos university hospital, muscat, oman, with bilateral pulmonary artery aneurysms and lowerlimb dvt who developed massive hemoptysis. Surgical resection provides an effective treatment option for patients with.
But the radiologic suspicions were of neoplastic lesions. We present the case of an 11yearold boy presenting with haemoptysis, dyspnoea and weight loss as a manifestation of isolated pulmonary vasculitis, leading to pulmonary hypertension. A previously healthy 23yearold saudi woman presented with massive hemoptysis a day prior to her admission to our hospital. We are reporting a 38yearold man with a history of right ventricular thrombus and also deep vein thrombosis who presented with massive hemoptysis. Hughes stovin syndrom metadata this file contains additional information such as exif metadata which may have been added by the digital camera, scanner, or software program used to create or digitize it. Hughes stovin syndrome is a rare condition first described in 1959 that is characterized by peripheral deep venous thrombosis dvt often involving the vena cava and accompanied by single or multiple pulmonary arterial aneurysms. Hughes stovin syndrome with pulmonary angiitis and focal glomerulonephritis chest. Hughes stovin syndrome is a lifethreatening disorder of unknown etiology.
A 25 year old male presented with optic nerve emboli. Characterized by multiple pulmonary artery aneurysms and peripheral venous thromboses, the hughes stovin syndrome is a rare and usually fatal disorder of unknown etiology which occurs almost exclusively in young males. Physical examination revealed no evidence of oral or genital ulcers. The syndrome s typical symptoms are recurrent cough, dyspnea, chest pain, fever, and hemoptysis.
Hughes stovin syndrome hss is an exceedingly rare and widely underrecognized phenomenon despite its high mortality rate 1. Multimodality imaging of hughesstovin syndrome european. This condition is characterized by vasculitis, deep venous thrombosis and aneurysms that mainly involve the pulmonary arteries resulting in hemoptysis. Hughes stovin syndrome is a very rare disease with fewer than 30 cases reported in the literature. We report the case of an 18 years old, greek male patient with hughes stovin syndrome, who initially presented with deep vein. Multiple pulmonary artery aneurysms and peripheral venous. From the department of internal medicine, division of cardiology and angiology, university of the saarland, homburg, germany m. Firstly, many antibiotic regimens that were tried during the treatment of hss were not effective. Hughes stovin syndrome hss was diagnosed on the basis of the association of multiple pulmonary arteries aneurysms, pulmonary embolism, and thrombus into the right atrium. Background hughes stovin syndrome is a rare condition first described in 1 which is characterized by multiple pulmonary artery aneurysms and peripheral venous thrombosis. Hughes stovin syndrome hss is a very rare clinical disorder characterized by thrombophlebitis and multiple pulmonary andor bronchial aneurysms.
The pathogenesis of hughes stovin syndrome is unknown. We report the case of a 33 years old man with no prior illnesses, who. The hemoptysis resolved with intravenous methylprednisolone treatment. Pdf hughesstovin syndrome hss is a very rare clinical disorder. The diagnosis of hughes stovin syndrome was made and the patient was treated with steroids and cyclophosphamide. Hughes stovin syndrome hss is a very rare clinical disorder with less than 40 cases published in the english literature so far 1. Successful closure of pulmonary artery aneurysm in a. Epidemiology it occurs predominantly between the 2nd to 4th decades. Less than 40 published cases of hss have been described in english medical literature so far. The disease is thought to be a variant of behcets disease and is defined by the presence of pulmonary artery aneurysm in association with peripheral venous thrombosis. An extraordinary cause in the etiology of recurrent massive. Case reports describing women with hughesstovin syndrome are rare. The main clinical manifestation is haemoptysis and dyspnea. A previously healthy 23yearold saudi woman presented with massive hemoptysis a day prior to her admission to our.
Hughesstovin syndrome orphanet journal of rare diseases full. A clinicopathologic case of hughes stovin syndrome with pulmonary eosinophilic angiitis and focal proliferative extracapillary glomerulonephritis is reported. It is characterized by a combination of systemic venous thrombosis and pulmonary artery aneurysm. Med any combination of signs and symptoms that are indicative of a particular disease or disorder syndrome, mr. This rare entity may be suspected when a relatively young patient presents with evidence of thromboe. Its considered as a clinical variant of behcets disease. Use of an amplatzer vascular plug in embolization of a. Hughesstovin syndrome hss is a very rare clinical disorder. Hughes stovin syndrome is a syndrome of unknown etiology characterized by thrombophlebitis and pulmonary aneurysms.
Hemoptysis caused by hughesstovin syndrome the american. The aetiology of hughesstovin syndrome is still unknown and the natural course of the illness is usually fatal. Massive hemoptysis and deep venous thrombosis presenting in a woman with hughesstovin syndrome. A rare cause of hemoptysis in patients with behcets disease. Hughes stovin syndrome is an extremely rare autoimmune clinical disorder that is character ized by deep vein thrombosis with multiple pulmonary andor bronchial arterial aneurysms. This syndrome is an important cardiovascular status that may manifest behcets disease with systemic aneurysms. Massive hemoptysis and deep venous thrombosis presenting. The eponym hughesstovin syndrome was applied by kopp and green in 1962. The pulmonary aneurysms of hughes stovin syndrome have almost always been diagnosed at autopsy, except the hughes stovin first case 1 and kopp and greens case, 5 where lobectomies have been done. The eponym hughes stovin syndrome was applied by kopp and green in 1962. This rare presentation, especially in childhood, might represent a case of the seldomly reported hughes stovin syndrome. Hughesstovin syndrome by umair khalid and taimur saleem.
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