Is hughesstovin syndrome a particular expression of. Hughesstovin syndrome hss, orpha228116 was named after two british physicians, drs. Treatment of hss includes immunosuppressants, which may regress. It is widely believed that lupus is a genetically determined disease. 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. Pdf hughesstovin syndrome hss is a very rare clinical disorder characterized by thrombophlebitis and multiple pulmonary andor bronchial aneurysms. Hughesstovin syndrome orphanet journal of rare diseases. 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 lifethreatening disorder, believed to be a. A patient is reported with pulmonary arterial occlusions and aneurysms and recurrent haemoptysis. 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. Have you suffered from a number of miscarriages but dont know why. Objectives to illustrate the use of infliximab in three cases. Hughesstovin syndrome definition of hughesstovin syndrome. Antiphospholipid syndrome also known as lupus anticoagulants or hughes syndrome kaleidoscope tips on how to properly deal with arthritis. 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. It also aids in assessment of angiodysplastic bronchial arteries in hss. We report the case of an 18 years old, greek male patient with hughes stovin syndrome, who initially presented with deep vein. The syndrome s typical symptoms are recurrent cough, dyspnea, chest pain, fever, and hemoptysis. Hughes stovin syndrome is a very rare pathology of unknown etiology with a lethal potential.
John patterson hughes and peter george ingle stovin. Less than 40 published cases of hss have been described in english medical literature so far. University of colorado health sciences center, denver. Successful closure of pulmonary artery aneurysm in a. Hughes stovin syndrome is a rare disorder of unknown etiology characterized by the association of multiple pulmonary artery aneurysms and deep venous thrombosis. Aps is one of the main causes of strokes 40%, leg deep vein thrombosis, and recurrent miscarriages in women.
Autoimmune disorders occur if the bodys immune system makes antibodies that attack and damage tissues or cells. Persistent fever with chills and an endocardial mass in a. Patients, mostly men aged 1240 years may present with haemoptysis, cough, dyspnea. Hughesstovin syndrome hss is a very rare clinical disorder characterized by thrombophlebitis and multiple pulmonary andor bronchial aneurysms. Background hughes stovin syndrome hss is described as a very rare vascular manifestation of behcets disease in medical literature. Hughes stovin syndrome is an exceedingly rare vasculitis, especially in childhood, consisting of multiple pulmonary artery aneurysms and deep venous thromboses. Hughesstovin syndrome article about hughesstovin syndrome. Ari provides patients with the opportunity to be a part of the solution when it comes to improving patient outcomes by filling out a simple, free survey. These findings may indicate a syndrome of obscure etiology. We present a case of a 27yearold man having a hughes stovin syndrome. Article in bulgarian georgieva e, pavlov k, nikolov s. Multiple, small paa warrant early immunosuppression.
A case of hughesstovin syndrome university of twente research. 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. It is a rare variant of behcets disease, which entails more general problems with the circulatory system. A treatment similar to that used in behcets disease, with isolate corticotherapy.
The aetiology of hughes stovin syndrome is still unknown and the natural course of the illness is usually fatal. Hughesstovin syndrome is a scarce entity associating pulmonary artery aneurysms and deep venous thrombosis. Hughesstovin syndrome is characterized by multiple pulmonary and. Patients affected by this syndrome are often young adult men in the second to fourth decade of life. In general, there is a predisposition for thrombus formation affecting the peripheral veins. I would like to express some remarks about this interesting case report. Are you prone to limb pain and circulation problems. Pdf hughesstovin syndrome is a rare disorder of unknown etiology characterized by the association of multiple pulmonary artery aneurysms and deep. The exact etiopathogenesis in hughes stovin syndrome is unrecognized 2 3. This condition is characterized by vasculitis, deep venous thrombosis and aneurysms that mainly involve the pulmonary arteries resulting in hemoptysis. The signs and symptoms of antiphospholipid antibody syndrome aps are related to abnormal blood clotting. Knowing that arthritis is a part of your life is stressful enough, and finding ways of dealing with it should not have to be stressful, as well. Hughes stovin syndrome synonyms, hughes stovin syndrome pronunciation, hughes stovin syndrome translation, english dictionary definition of hughes stovin syndrome. Table 3 causes for pulmonary artery aneurysms without arteriovenous communication adapted from fischer et al 18.
Pulmonary artery aneurysms paa have a diverse differential diagnosis. Combined pulmonary vasodilator therapy and endovascular. Med any combination of signs and symptoms that are indicative of a particular disease or disorder syndrome, mr. Hughes stovin syndrome is a syndrome of unknown etiology characterized by thrombophlebitis and pulmonary aneurysms. Massive hemoptysis and deep venous thrombosis presenting in a woman with hughesstovin syndrome. Immunosuppressive therapy is the most common treatment, involving a mix of glucocorticoids and cyclophosphamide. 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. 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. It is named after the two british physicians, john patterson hughes and peter george ingle stovin, who first described it in 1959. There is no standard treatment for hughesstovin syndrome, the most widely used treatment option being immunosuppression therapy involving a combination of. Hughes stovin syndrome is a scarce entity associating pulmonary artery aneurysms and deep venous thrombosis. 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.
Antiphospholipid syndrome addresses the complete range of conditions produced by this common disorder which is also known as aps or sticky blood syndrome. Blood clots can form in, or travel to, the arteries or veins in the brain, heart, kidneys, lungs, and limbs. Less than 40 published cases of hss have been described in. This article is within the scope of wikiproject medicine, which recommends that medicinerelated articles follow the manual of style for medicinerelated articles and that biomedical information in any article use highquality medical sources. Successful treatment of refractory hughes stovin syndrome. Multimodality imaging of hughesstovin syndrome european. Hughesstovinsyndrom1960 ursachlich unklares krankheitsbild mit wiederkehrenden oberflachlichen polytopen thrombophlebitiden evtl. The patient had a history of left iliofemoral thrombosis and suffered from recurrent oral ulcers. Likewise, in our case,nosaphenousveinthrombosiswas found. Married to kent hughes, pastor of college church in wheaten, illinois, she is the author of disciplines of a godly woman and coauthor with her husband of liberating ministry from the success syndrome and common sense parenting. Successful treatment of refractory hughes stovin syndrome with infliximab successful treatment of refractory hughes stovin syndrome with infliximab.
Stovin syndrome and was the first female case of typical hughes. Earlier diagnosis could be in some cases crucial, allowing the prevention of the development of potentially lifethreatening pulmonary artery aneurysms 9. Pulmonary medium vessel vasculitis in an 11 year old boy. It was considered to be a form of behcets syndrome at its initial feature. Hughes stovin syndrome is a rare clinical condition characterized by multiple pulmonary artery aneurysms and peripheral venous thrombosis, first described in 1. Introduction hughes stovin syndrome is a rare pathology associating pulmonary artery aneurysms and deep vein thrombosis and affecting commonly the young patient with a masculine predominance 1 2. Hughesstovin syndrom unvollstandige auspragung des. Hughes stovin syndrome hss is a very rare clinical disorder characterized by thrombophlebitis and multiple pulmonary andor bronchial aneurysms.
It is characterized by a combination of systemic venous thrombosis and pulmonary artery aneurysm. Less than 40 cases have ever been reported in the english medical literature. Massive pulmonary hemorrhage caused by rupture of an aneurysm is a frequent terminal event. A case of hughesstovin syndrome incomplete behcets disease. Bronchoscopy is often done in hss patients who present with hemoptysis. Most patients are young adult males between the age of 2040. What chronic illnesses can affect fertility and why. Hughesstovin syndrome is a rare clinical condition characterized by multiple pulmonary artery aneurysms and peripheral venous thrombosis, first described in 1959. I was diagnosed with hughes syndrome after several recurrent miscarriages between 1012 weeks and the only time i have taken medication has been dur. The aetiology of hughesstovin syndrome is still unknown and the natural course of the illness is usually fatal. Walker2 from the 1department of pediatrics, mcmaster university, hamilton, on l8n 3z5, canada, 2department of cardiothoracic surgery and 3department of renal medicine, royal in. Hughes stovin 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 life711. 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.
Please visit the project page for details or ask questions at wikipedia talk. False aneurysm of the pulmonary artery with peripheral venous thrombosis. Hemoptysis, pulmonary aneurysm, hughes stovin syndrome 1. They usually help defend the body against infections. It has been described in literature less than 40 times. Background hughesstovin syndrome is a rare condition first described in 1 which is characterized by multiple pulmonary artery aneurysms and 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. The response to treatment was very good and at the 12 months followup the patient was completely asymptomatic, with no new radiological. 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 friends of pdf all our visitors are our friends. We report the case of an 18 years old, greek male patient with hughesstovin syndrome, who initially presented with deep vein thrombosis. Chronic illnesses are stressful for the body and some conditions, especially if they cause weight loss, can interfere with ovulation. One described regimen for the treatment of arterial aneurysms is monthly pulses of cyclophosphamide 1 gram plus prednisolone 1 mgkgday. Hughes stovin syndrome is a lifethreatening disorder of unknown etiology. Life threatening hemoptysis from hughes stovin syndrome.
Antiphospholipid antefosfolipid antibody syndrome aps is an autoimmune disorder. The exact etiology and pathogenesis of hss is unknown. In the patient presented in this report they were associated with recurrent thrombosis of systemic veins and dural sinuses. He gave a history of recurrent arthropathy and febrile illnesses. It is characterized by the association of pulmonary artery aneurysms and peripheral venous thrombosis. If you do not see its contents the file may be temporarily unavailable at the journal website or you do not have a pdf plugin installed and enabled in your browser. Hss is a very rare clinical disorder and around 4060 published cases of hss have been described in medical literature so far. Hughesstovin syndrome hss, a rare syndrome considered by some.
Although most are asymptomatic at diagnosis, fatal aneurysm rupture occurs in onethird and dissection in onefifth. Hughes stovin syndrome hss, characterized by the combination of multiple pulmonary artery aneurysms and deep vein thrombosis, is a rare and an underrecognized clinical entity with less than 40 published cases in english medical literature. Hughes stovin syndrome hss is a very rare clinical disorder with less than 40 cases published in the english literature so far 1. To submit an update or takedown request for this paper, please submit an updatecorrectionremoval request. Barbara hughes has many years of experience as a pastors wife. Our goal is to be the goto resource for autoimmune disease. Surgical resection provides an effective treatment option for patients with. Some authors consider this entity an incomplete form of behcets disease due to the similarities between the radiologic and anatomopathological findings of pulmonary involvement. 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. We report the case of a 33 years old man with no prior illnesses, who. Hughesstovin syndrome, pulmonary artery aneurysms and deep. We are reporting a 38yearold man with a history of right ventricular thrombus and also deep vein thrombosis who presented with massive hemoptysis. The outcome of a blood clot depends on its size and location. If so, than you might be suffering from hughes syndrome, an immune system disorder that can threaten the health of both you and your baby.
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