Hughes stovin syndrome hss, orpha228116 was named after two british physicians, drs. Less than 40 published cases of hss have been described in english medical literature so far. Hughes syndrome is named after dr graham hughes, who along with his team in london, was the first to describe the condition in 1983. Hughes syndrome, also known as antiphospholipid syndrome or sticky blood is a disorder characterized by blood clotting, both in the arteries and veins.
Hughesstovin syndrome definition of hughesstovin syndrome. Pdf hughesstovin syndrome is a rare disorder of unknown etiology characterized by the association of multiple pulmonary artery aneurysms and deep. Hughesstovin syndrome is a rare autoimmune disorder of unknown cause that is characterized by the combination of multiple pulmonary artery aneurysms and. 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. Cureus hughes stovin syndrome, a rare form of behcets. He would fulfill the criteria for hughes stovin syndrome, which was initially described in 1959 and defined as a syndrome of multiple pulmonary artery aneurysms with extensive venous thrombosis. Intracardiac thrombus occurring as a thrombotic manifestation of hss is an unusual presentation and represents a challenge in.
Hughesstovin syndrome, pulmonary artery aneurysms and deep venous thrombosis. Apr, 2011 hughes stovin syndrome hss is a very rare clinical disorder characterized by thrombophlebitis and multiple pulmonary andor bronchial aneurysms. Hughes stovin syndrome synonyms, hughes stovin syndrome pronunciation, hughes stovin syndrome translation, english dictionary definition of hughes stovin syndrome. Pdf hughesstovin syndrome hss is a rare autoimmune disorder, characterized. Aps provokes blood clots in both arteries and veins as well as pregnancyrelated complications such as miscarriage, stillbirth, preterm delivery, and severe preeclampsia. The exact etiology and pathogenesis of hss is unknown. Hughes syndrome is caused by the presence of antibodies in the blood, called antiphospholipid antibodies. Life threatening hemoptysis from hughes stovin syndrome.
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. This condition is characterized by vasculitis, deep venous thrombosis and aneurysms that mainly involve the pulmonary arteries resulting in hemoptysis. Treatment is currently based on immunosuppression and oral. 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.
Behcet syndrome, hughesstovin syndrome, pulmonary artery aneurysm, vasculitis. Hughes syndrome an autoimmune condition caused by an antibody to phospholipid, a major constituent of cell plasma membranes, or an antibody to cardiolipin. Hughesstovin syndrome synonyms, hughesstovin syndrome pronunciation, hughesstovin syndrome translation, english dictionary definition of hughesstovin syndrome. Arterial blood culture repeatedly examined had been negative.
Stovin syndrome is an exceedingly rare disorder consisting of multiple pulmonary aneurysms and peripheral venous thrombosis. He would fulfill the criteria for hughesstovin syndrome, which was initially described in 1959 and defined as a syndrome of multiple pulmonary artery aneurysms with extensive venous thrombosis. Pulmonary artery aneurysms with recurrent thrombophlebitis. Pulmonary artery aneurysms paa are uncommon, with a diverse differential diagnosis. Massive hemoptysis and deep venous thrombosis presenting in a. There is no standard treatment for hughesstovin syndrome, the most widely used treatment option being immunosuppression therapy involving a combination of. Incredibles wannabe sidekick turned bad guy, from the incredibles 2004. Intracardiac thrombus occurring as a thrombotic manifestation of hss is an unusual presentation and represents a challenge in diagnosis and. Hughesstovin syndrome article about hughesstovin syndrome. The hughesstovin syndrome consists of deep venous thrombosis often involving the caval vein accompanied by single or multiple. The hughes stovin syndrome consists of deep venous thrombosis often involving the caval vein accompanied by single or multiple pulmonary arterial aneurysms.
A group of symptoms that collectively indicate or characterize a disease, disorder, or other condition considered abnormal. Jan 29, 2009 hughesstovin syndrome is a rare clinical condition characterized by multiple pulmonary artery aneurysms and peripheral venous thrombosis, first described in 1959. 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. The true existence of this syndrome as a distinct clinical entity has been much debated, with some authors favoring hughes stovin as a variant of the. Hughes syndrome definition of hughes syndrome by medical. Overview hughesstovin syndrome is a rare clinical condition characterized by multiple pulmonary artery aneurysms and peripheral venous thrombosis, first described in 1959 1. Severe cardiovascular involvement in hughesstovin syndrome. Hughes stovin syndrome is a rare condition characterized by peripheral deep venous thrombosis accompanied by single or multiple pulmonary arterial aneurysms. The first patient was a 26yearold male who initially presented with deep vein thrombosis dvt in the right lower limb followed shortly by 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. Review open access 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. 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. Hughesstovin syndrome hss is a very rare clinical disorder.
Idiopathic and vascular diseases of the thorax have been the subject of recent publications in the radiology literature of brazil 16. Hughes stovin syndrome is a lifethreatening disorder of unknown etiology. Hughes syndrome foundation nord national organization. The hughes syndrome foundations hsf mission is to provide information and education on hughes syndrome and support research for it. Multiple pulmonary artery aneurysms and peripheral venous. The clinical presentation may include cough or hemoptysis and venous thromboses either in the extremities, the dural sinuses, the vena cavae. Hughesstovin syndrome is a rare clinical condition characterized by multiple pulmonary artery aneurysms and peripheral venous thrombosis, first described in 1959. Antiphospholipid antibody syndrome aps has no cure. It is named after the two british physicians, john patterson hughes and peter george ingle stovin, who first described it in 1959. Hughes stovin syndrome hss is a particularly rare disease characterized by multiple pulmonary artery andor bronchial artery aneurysms with concomitant peripheral venous thrombosis and is believed to be a cardiovascular variant of behcets disease. Since its first description by hughes and stovin in 1959, there have been free at 18 months with immunosuppression. Hughesstovin syndrome is a very rare clinical entity characterized by pulmonary artery aneurysms and deep vein thrombosis dvt.
Radiologic findings in the diagnosis of hughesstovin syndrome. A patient is reported with pulmonary arterial occlusions and aneurysms and recurrent haemoptysis. Hughesstovin syndrome is a rare condition, characterized by the combination of multiple pulmonary artery aneurysms and peripheral venous thrombosis, that mainly affects males 8090% of cases between the second and fourth decades of life 711. Massive pulmonary hemorrhage caused by rupture of an aneurysm is a frequent terminal event. 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. The disease bears some resemblance to behcet disease. Also known as sticky blood, it is an autoimmune disorder, in which there is a danger of thrombosis clotting. Sep 04, 2019 hughes stovin syndrome pdf september 4, 2019 0 comment hughesstovin syndrome is a rare autoimmune disorder of unknown cause that is characterized by the combination of multiple pulmonary artery aneurysms and. The limited number of cases has precluded controlled studies of the management of pulmonary artery aneurysms, which usually cause massive hemoptysis leading to death. Hughesstovin syndrome is an exceedingly rare vasculitis, especially in childhood, consisting of multiple pulmonary artery aneurysms and deep venous thromboses. The hughesstovin syndrome consists of deep venous thrombosis often involving the caval vein accompanied by single or multiple pulmonary arterial aneurysms.
One described regimen for the treatment of arterial aneurysms is monthly. 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. Access to this free content requires users to be registered and logged in. Pulmonary artery aneurysms with recurrent thrombophlebitisthe. The goals of treatment are to prevent blood clots from forming and keep existing clots from getting larger. 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. The true existence of this syndrome as a distinct clinical entity has been much debated, with some authors favoring hughesstovin as a variant of the. Patients, mostly men aged 1240 years may present with haemoptysis, cough, dyspnea, chest pain, and signs of pulmonary hypertension. Hsf offers understanding and support to sufferers of hughes syndrome which is known medically as antiphospholipid syndrome aps. This means people with aps are at greater risk of developing conditions such as. Epidemiology it occurs predominantly between the 2nd to 4th decades. Persistent fever with chills and an endocardial mass in a. Patients affected by this syndrome are often young adult men in the second to fourth decade of life.
Hughesstovin syndrome is a rare clinical condition characterized by multiple pulmonary artery aneurysms and peripheral venous thrombosis, first described in 1959 1. The lack of formal diagnostic criteria and the rarity of the disease make the diagnosis very challenging, especially when respiratory complaints are not present at onset, as in the. Aps is a rare immunological disorder characterized by recurring blood clots. Antiphospholipid syndrome, or antiphospholipid antibody syndrome aps or apls, is an autoimmune, hypercoagulable state caused by antiphospholipid antibodies.
Hughesstovin syndrome is a lifethreatening disorder of unknown etiology. Pdf hughesstovin syndrome is a rare disorder of unknown etiology. Hughes stovin syndrome hss is a very rare clinical disorder characterized by thrombophlebitis and multiple pulmonary andor bronchial aneurysms. You may have aps and another autoimmune disorder, such as lupus. Hughes stovin syndrome is a very rare clinical entity characterized by pulmonary artery aneurysms and deep vein thrombosis dvt. Hughes stovin syndrome as a variant of behcet disease associated with a right atrial myxoma. Hughesstovin syndrome hss is a very rare clinical disorder characterized by thrombophlebitis and multiple pulmonary andor bronchial aneurysms. This is the first report of a new endovascular treatment of a single. She underwent multiple investigations elsewhere and was put on antituberculosis treatment. Syndrome pop culture when innocent heroworship goes unrequited, the consequences can be dire for both the admirer and the admired.
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. In this report, we described two male patients with hughesstovin syndrome. Hughes stovin syndrome as a variant of behcet disease associ. Hughes stovin syndrome information including symptoms, causes, diseases, symptoms, treatments, and other medical and health issues. It features recurrent thromboses causing strokes, heart attacks and other arterial obstruction effects, or recurrent fetal loss during pregnancy. Firstly, many antibiotic regimens that were tried during the treatment of hss were. Hughesstovin syndrome is a rare disorder of unknown etiology characterized. Antiphospholipid syndrome aps, sometimes known as hughes syndrome, is a disorder of the immune system that causes an increased risk of blood clots.
If the address matches an existing account you will receive an email with instructions to reset your password. 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 rare disorder, first described in 1959 by j p. Apr 21, 2010 hughes stovin syndrome is a very rare disease with fewer than 30 cases reported in the literature. She symptom free at 18 months with immunosuppression.
Hughesstovin syndrome orphanet journal of rare diseases. Hughes stovin syndrome is a rare clinical condition characterized by multiple pulmonary artery aneurysms and peripheral venous thrombosis, first described in 1959. Stovin syndrome and was the first female case of typical hughes. Hughesstovin syndrome europe pmc article europe pmc. 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. Pulmonary artery aneurysms paa have a diverse differential diagnosis.
Hughesstovin syndrome information including symptoms, causes, diseases, symptoms, treatments, and other medical and health issues. Although most are asymptomatic at diagnosis, fatal aneurysm rupture occurs in onethird and dissection in onefifth. Pulmonary ct angiography revealed bilateral pulmonary artery aneurysms secondary to underlying pulmonary vasculitis. Hughesstovin syndrome hss is a vasculitis that predominantly affects large vessels. It has been described in literature less than 40 times. Stovin, 1 with fewer than 40 cases reported worldwide2, 3 and most commonly affecting young men aged 1240 years. New approach in the diagnosis of and therapy for hughes stovin syndrome. Apr 25, 2007 in this report, we described two male patients with hughesstovin syndrome. A 25yearold woman was found to have hughes stovin syndrome, previously reported only in males. Use of an amplatzer vascular plug in embolization of a. He gave a history of recurrent arthropathy and febrile illnesses. John patterson hughes and peter george ingle stovin.