Hughes stovin syndrome pdf

Hughesstovin syndrome hss is a raredisorder of unknown etiology. 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. Less than 40 published cases of hss have been described in english medical literature so far. Hughesstovin syndrome orphanet journal of rare diseases.

The aetiology of hughesstovin syndrome is still unknown and the natural course of the illness is usually fatal. The eponym hughesstovin syndrome was applied by kopp and green in 1962. Multiple pulmonary andor bronchial aneurysms as well as recurrent phlebitis frequently involving the large vessels resulting in thromboembolism are characteristics of this syndrome. Hughesstovin syndrome definition of hughesstovin syndrome. Although most are asymptomatic at diagnosis, fatal aneurysm rupture occurs in onethird and dissection in onefifth. A rare cause of hemoptysis in patients with behcets disease. This rare entity may be suspected when a relatively young patient presents with evidence of thromboe. Hughesstovin syndrome is a very rare disease with fewer than 30 cases reported in the literature.

Hughesstovin syndrome umair khalid and taimur saleem abstract hughesstovin syndrome hss is a very rare clinical disorder characterized by thrombophlebitis and multiple pulmonary andor bronchial aneurysms. Hughesstovin syndrome with pulmonary angiitis and focal. A rare cause of hemoptysis in patients with behcets. The diagnosis of hughesstovin syndrome was made and the patient was treated with steroids and cyclophosphamide.

Pulmonary artery aneurysms paa have a diverse differential diagnosis. These findings may indicate a syndrome of obscure etiology. The hughesstovin syndrome consists of deep venous thrombosis often involving the caval vein accompanied by single or multiple pulmonary arterial aneurysms. Hughesstovin syndrome hss is a lifethreatening disorder, believed to be a cardiovascular clinical variant manifestation of behcets disease bd. Hughesstovin syndrome is a rare autoimmune disorder of unknown cause that is. Hughesstovin syndrome is a rare disorder of unknown etiology characterized by the association of multiple pulmonary artery aneurysms and deep venous thrombosis. Weber aa hughesstovin syndrome is a rare condition first described in 1959 1 and is characterized by multiple pulmonary aneurysms and peripheral venous thrombosis. An extraordinary cause in the etiology of recurrent massive.

Massive hemoptysis and deep venous thrombosis presenting. 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. It is a rare variant of behcets disease, which entails more general problems with the circulatory system. Hughes stovin syndrome shs is a pathology of unknown etiology with a lethal potential. It is named after the two british physicians, john patterson hughes and peter george ingle stovin, who first described it in 1959. Hughesstovin syndrome complicated with fatal pneumothorax. Review of the literature revealed 9 typical cases of this syndrome and 5 atypical cases who had solitary intrapulmonary. The pathogenesis of hughes stovin syndrome is unknown. Hughesstovin syndrome hss is a vasculitis that predominantly affects large vessels. This syndrome was named in honor of two english doctors dr.

We report the case of a 33 years old man with no prior illnesses, who. Ct angiography of pulmonary artery aneurysms in hughes. Pdf hughesstovin syndrome is a rare disorder of unknown etiology characterized by the association of multiple pulmonary artery aneurysms and deep. An extraordinary cause in the etiology of recurrent. A patient is reported with pulmonary arterial occlusions and aneurysms and recurrent haemoptysis. He presented with pulmonary arterial aneurysms figure 1 thrombosis of the hepatic and inferior vena cava veins and recurrent bipolar aphthosis. Jun 14, 2019 hughesstovin syndrome is a rare autoimmune disorder of unknown cause that is characterized by the combination of multiple pulmonary artery aneurysms and.

Antiphospholipid syndrome, or antiphospholipid antibody syndrome aps or apls, is an autoimmune, hypercoagulable state caused by antiphospholipid antibodies. 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. 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. Hughesstovin syndromehss is an exceedingly rare and widely underrecognized phenomenon despite its high mortality.

Hughesstovin syndrome article about hughesstovin syndrome. 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. Hughes stovin syndrome is a rare autoimmune disorder of unknown cause that is characterized by the combination of multiple pulmonary artery aneurysms and deep vein thrombosis. From the department of internal medicine, division of cardiology and angiology, university of the saarland, homburg, germany m. The main clinical manifestation is haemoptysis and dyspnea. It is characterized by the association of pulmonary artery aneurysms and peripheral venous thrombosis. Hughesstovin syndrome hss is a very rare clinical disorder characterized by thrombophlebitis and multiple pulmonary andor bronchial aneurysms. Hughesstovin syndrome is a very rare clinical entity characterized by pulmonary artery aneurysms and deep vein thrombosis dvt. They first described the findings of the syndrome deep venous thrombosis and segmental pulmonary artery aneurysms in a total of four male patients with pulmonary artery aneurysms in 1959 1. This syndrome is an important cardiovascular status that may manifest behcets disease with systemic 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.

But the radiologic suspicions were of neo plastic lesions. In 1959, the two doctors described for the first time the syndrome as the association between deep vein thrombosis. Weber aa hughes stovin syndrome is a rare condition first described in 1959 1 and is characterized by multiple pulmonary aneurysms and peripheral venous thrombosis. When i read the case report by meireles and colleagues about hughes stovin syndrome with pulmonary angiitis and focal glomerulitis chest 1981. Hughesstovin syndrome is an exceedingly rare vasculitis, especially in childhood, consisting of multiple pulmonary artery aneurysms and deep venous thromboses. Our report underlines the importance of tnf blocker treatment in hughes stovin syndrome, suggesting its use as long. Hughes stovin syndrome hss is a raredisorder of unknown etiology. The management of hss can either be medical or surgical. Although tuberculosis is the most common cause for hemoptysis in tropical countries, we need to rule out very rare causes such as behcet syndrome and hughesstovin syndrome. It is characterized by a combination of systemic venous thrombosis and pulmonary artery aneurysm. The hughes stovin syndrome consists of deep venous thrombosis often involving the caval vein accompanied by single or multiple pulmonary arterial aneurysms.

Hughesstovin syndrome is an extremely rare autoimmune clinical disorder that is character ized by deep vein thrombosis with multiple pulmonary andor bronchial arterial aneurysms. 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. Multimodality imaging of hughesstovin syndrome european. Some authors consider this entity an incomplete form of behcets disease due to the similarities between the radiologic and anatomopathological findings of pulmonary involvement. The disease bears some resemblance to behcet disease.

The use of a pathergy test that has been accepted as one of the major criteria in behcets disease shows a negative finding for hughesstovin syndrome. 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. Abstract hughesstovin syndrome hss is a very rare clinical disorder characterized by thrombophlebitis and multiple pulmonary andor bronchial aneurysms. Typical symptoms are recurrent fever, chills, haemoptysis and coughs.

Hughesstovin syndrome hss, orpha228116 was named after two british physicians, drs. Stovin syndrome and was the first female case of typical hughes. Hughes stovin syndrome hss is a very rare clinical disorder characterized by thrombophlebitis and multiple pulmonary andor bronchial aneurysms. Although the association between multiple pulmonary artery aneurysms and venous thrombosis of the lower limbs had been reported by beattie and hall in 1911, it was not until 1962 that the eponym hughesstovin syndrome was formally introduced in medical literature for hss. Hughesstovin syndrome hss is a very rare condition characterized by pulmonary aneurysms and peripheral thrombophlebitis of lower limbs. Hughesstovin syndrome without venous involvement unusual. Characterized by multiple pulmonary artery aneurysms and peripheral venous thromboses, the hughesstovin syndrome is a rare and usually fatal disorder of unknown etiology which occurs almost exclusively in young males. Oct 06, 2019 hughes stovin syndrome pdf october 6, 2019 admin hughesstovin syndrome is a rare autoimmune disorder of unknown cause that is characterized by the combination of multiple pulmonary artery aneurysms and. Treatment for the paas is mainly the use of immunosuppressive agents such as steroids, cyclophosphamide or azathioprine. Patients, mostly men aged 1240 years may present with haemoptysis, cough, dyspnea, chest pain, and signs of pulmonary hypertension. Is hughesstovin syndrome a particular expression of behcets.

Hughesstovin syndrome hss is a very rare clinical disorder characterized by thrombophlebitis and multiple pulmonary andor bronchial. Multiple pulmonary artery aneurysms and peripheral venous. John patterson hughes and peter george ingle stovin. This condition is characterized by vasculitis, deep venous thrombosis and aneurysms that mainly involve the pulmonary arteries resulting in hemoptysis. Being an extremely rare disease, there is no formally described diagnostic criteria or pathognomonic laboratory investigation for this syndrome. Hughes stovin syndrome synonyms, hughes stovin syndrome pronunciation, hughes stovin syndrome translation, english dictionary definition of hughes stovin syndrome. Objectives to illustrate the use of infliximab in three cases. The typical clinical presentation of hughesstovin syndrome consists of three phases. Hughesstovin syndrome hss is a very rare clinical disorder with less than 40 cases published in the english literature so far 1.

Hughesstovin syndrome is a rare clinical condition characterized by multiple pulmonary artery aneurysms and peripheral venous thrombosis, first described in 1959 1. Is hughesstovin syndrome a particular expression of. Jan 29, 2009 hughesstovin syndrome is a rare entity. Apr, 2011 hughes stovin syndrome hss is a very rare clinical disorder characterized by thrombophlebitis and multiple pulmonary andor bronchial aneurysms. Hughesstovin syndrome hss is a very rare clinical disorder characterized by thrombophlebitis and multiple pulmonary aneurysms. A 25yearold woman was found to have hughes stovin syndrome, previously reported only in males. Hughesstovin syndrome is a scarce entity associating pulmonary artery aneurysms and deep venous thrombosis. Life threatening hemoptysis from hughes stovin syndrome. The hughesstovin syndrome rsna publications online.

We report the case of an 18 years old, greek male patient with hughesstovin syndrome, who initially presented with deep vein thrombosis. Hughesstovin syndrome is an exceedingly rare vasculitis, especially in childhood, consisting of multiple pulmonary artery aneurysms and. It is characterized by the association of multiple pulmonary artery aneurysms paas and peripheral venous thrombosis. It was considered to be a form of behcets syndrome at its initial feature. Hughes stovin syndrome pdf hughesstovin syndrome is a rare autoimmune disorder of unknown cause that is characterized by the combination of. Massive pulmonary hemorrhage caused by rupture of an aneurysm is a frequent terminal event. A 25 year old male presented with optic nerve emboli.

Pdf hughesstovin syndrome hss is a very rare clinical disorder characterized by thrombophlebitis and multiple pulmonary andor bronchial aneurysms. Some authors consider this entity an incomplete form of behcets disease. Hughesstovin syndrome is a lifethreatening disorder of unknown etiology. Hughes stovin syndrome hss is a lifethreatening disorder of unknown etiology. Hss is a very rare clinical disorder and around 4060 published cases of hss have been described in medical literature so far. Percutaneous transhepatic venous embolization of pulmonary. Arterial blood culture repeatedly examined had been negative.

Observation a 21yearold man was followed for six months in the internal medicine department for hughesstovin syndrome. The hughesstovin syndrome is a rare entity of uncertain etiology, consisting of one or more pulmonary artery aneu rysms, pulmonary emboli or thrombi, and. Med any combination of signs and symptoms that are indicative of a particular disease or disorder syndrome, mr. I would like to express some remarks about this interesting case report. Hughesstovin syndrome is a rare disorder of unknown etiology characterized by the association of multiple pulmonary artery aneurysms and deep venous. Aps provokes blood clots in both arteries and veins as well as pregnancyrelated complications such as miscarriage, stillbirth, preterm delivery, and severe preeclampsia. Its considered as a clinical variant of behcets disease.

A 25yearold woman was found to have hughesstovin syndrome, previously reported only in males. Patients affected by this syndrome are often young adult men in the second to fourth decade of life. The exact etiology and pathogenesis of hss is unknown. He gave a history of recurrent arthropathy and febrile illnesses. Physical examination revealed no evidence of oral or genital ulcers. Only after the late appearance of radiological pulmonary lesions and recognition of pulmonary artery aneurysms, hughesstovin syndrome was diagnosed. Persistent fever with chills and an endocardial mass in a. Less than 40 published cases of hss have been described in. Hughesstovin syndrome have almost always been diag nosed at autopsy, except the hughesstovin first easel and kopp and greens case, where lobectomies have been done. This condition is usually presented by vasculitis, deep venous thrombosis and aneurysms that usually involve the pulmonary arteries as well as the bronchial arteries resulting in hemoptysis. Hughesstovin syndrome europe pmc article europe pmc. The etiopathogenesis of hughesstovin syndrome remains unexplained. Hughesstovin syndrome is a very rare pathology of unknown etiology with a lethal potential. Hughesstovin syndrome hss is a lifethreatening disorder, believed to be a.