Rare case of multiple pulmonary artery aneurysms with caval thrombosis — Hughes-Stovin syndrome

Kim, Jung Tae; Oh, Tae Yun; Chang, Woon Ha

doi:10.1016/j.ejcts.2006.12.005

Cited by 18 publications

(13 citation statements)

References 8 publications

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“…The population-based incidence of HSS cannot be exactly determined. It usually affects young adults, especially males [6,7]. Being an extremely rare disease, there are no formally described diagnostic criteria or pathognomonic laboratory investigations for this syndrome.…”

Section: Discussionmentioning

confidence: 99%

A case of Hughes–Stovin syndrome (incomplete Behçet’s disease) with extensive arterial involvement

et al. 2019

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A case of Hughes-Stovin syndrome (incomplete Behçet's disease) with extensive arterial involvement Unmasking the true face of a rare syndrome Robinson et al. [16] Case 4: A 21-year-old male; recurrent oral ulcers; no genital ulceration; superficial thrombophlebitis; pulmonary CT angiography a 35 mm right lateral segmental PAA with multiple pulmonary artery in situ thromboses Demirkan and Gültekin [17] Case 5, 6: two patients with HSS who presented with pulmonary artery aneurysm, thrombophlebitis, hemoptysis, and oral ulcers Al-Jahdali [18] Case 7: 23-year-old Saudi woman; recurrent oral ulceration; right-lower lobe PAA; papilledema; DVT Yagi et al. [19] Case 8: A 32-year-old male; multiple PAA; DVT of the right leg and the right femoral vein; thrombosis of the vena cava; aphthous ulcer in the oral cavity, an ulcer in the genital region Madiha and Sami [20] Case 9: DVT; oral ulcers; giant aneurysm in the left lower lobe pulmonary artery Summary of HSS case reports without features of the classic triad of BD Hughes and Stovin [1] Case 1, 2: two male patients; segmental PPA with peripheral venous thrombosis Kopp and Green [21] Case 3, 4: two male patients; PAA and recurrent thrombophlebitis Fabi et al. [22] Case 5: A 12-year-old boy; right atrium endocardial mass; jugular vein and cerebral venous thrombosis; deep venous thromboses; PAA Abdelbary et al. [23] Case 6: A 35-year-old Egyptian female lower limb deep vein thrombosis; pulmonary aneurysm Ribeiro et al. [24] Case 7: A 43-year-old male; superficial thrombophlebitis and DVT of the lower limbs; PAA Pankl et al. [25] Case 8: A 41-year-old man; deep venous thrombosis of the right leg, and PAA Al-Zeedy et al. [26] Case 9: A 53-year-old man; DVT and PAA Kably and Reveron [27] Case 10: A 41-year-old male; massive hemoptysis; ruptured PAA; DVT; cardio-venous thromboembolism; pulmonary infarction El Aoud et al. [28] Case 11: A 42-year-old woman; DVT; PAA Jaramillo et al. [29] Case 12: A 47-year-old male; dilated main pulmonary arteries, multiple right bronchial artery aneurysms and a splenic artery aneurysm Silva et al. [30] Case 13: A 25-year-old male; DVT; PAA Grembiale et al. [31] Case 14: DVT; PAA; Budd-Chiari syndrome; a thrombotic occlusion of inferior vena cava Amezyane et al. [32] Case 15: A 28-year-old female; right ventricular thrombus, PAA, iliac vein thrombosis; caval thrombosis Kim et al. [7] Case 16: A 45-year-old man; massive hemoptysis; DVT; bilateral PAA and inferior vena caval thrombosis Chalazonitis et al. [9] Case 17: A 18-year-old, Greek male patient; DVT; PAA; superior sagittal and transverse sinuses Emad et al. [2] Case 18, 19: two male patients; DVT, PAA, superior sagittal sinus thrombosis in one case Balci et al. [33] Case 20: A 41-year-old patient; multiple PAA; thrombus in both the inferior and superior vena cavae Herb et al. [34] Case 21: A 25-year-old man; PAA; multiple aneurysms of the bronchial arteries; severe hemoptysis; aneurysm of the left hepatic artery Margolesky et al. [35] Case 22: A 38-year-old woman; DVT, PAA; right ventricle th...

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Section: Discussionmentioning

confidence: 99%

A case of Hughes–Stovin syndrome (incomplete Behçet’s disease) with extensive arterial involvement

et al. 2019

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“…HSS does not appear to have preponderance for any geographic location. Cases of HSS have been reported from diverse geographical areas including North America, Europe, Africa and Asia [11-14]. None of the reports have mentioned consanguineous marriages in the parents of the patients suffering from HSS.…”

Section: Epidemiologymentioning

confidence: 99%

“…Firstly, various antibiotic regimens that have been tried in the treatment of HSS have proven ineffective. Secondly, there has been a lack of positive blood cultures in the evaluation of patients with HSS [1-3,11,29,31,32,34,35]. Cultures of other body fluids in patients have also been found to be sterile [2].…”

Section: Etiology and Pathogenesismentioning

confidence: 99%

Hughes-Stovin Syndrome

Khalid

Saleem

2011

Orphanet Journal of Rare Diseases

145

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Hughes-Stovin Syndrome (HSS) is a very rare clinical disorder characterized by thrombophlebitis and multiple pulmonary and/or bronchial aneurysms. Less than 40 published cases of HSS have been described in English medical literature so far. The exact etiology and pathogenesis of HSS is unknown; possible causes include infections and angiodysplasia. HSS has also been considered as a variant of Behcet's disease (BD). Patients with HSS usually present with cough, dyspnea, fever, chest pain and haemoptysis. The management of HSS can either be medical or surgical. Medical management includes the use of steroids and cytotoxic agents. Cyclophosphamide, in particular, is a favored therapeutic agent in this regard. Antibiotics have no proven role in HSS while anticoagulants and thombolytic agents are generally contraindicated due to an increased risk of fatal hemorrhage. However, their use may be considered with great care under special circumstances, for instance, intracardiac thrombi or massive pulmonary embolism. For cases of massive hemoptysis due to large pulmonary aneurysms or those with lesions confined to one segment or one lung, lobectomy or pneumectomy can be carried out. However, surgical risks merit serious consideration and must be discussed with the patient. Transcatheter arterial embolization has emerged as a less invasive alternative to surgery in selected cases of HSS. Overall, patients with HSS have a poor prognosis and aneurysmal rupture is the leading cause of death. However, early diagnosis and timely intervention is crucial in improving the prognosis. There is a need to clearly elucidate the genetic, etiologic and pathologic basis for HSS in the future. Although most of the evidence put forward to refute the role of an infectious agent in the etiology and pathogenesis of HSS is based on negative blood and other body fluid cultures, more robust objective assessment is needed through the use of electron microscopy or 16 sRNA studies. The development of better therapeutic agents is also needed to address and prevent the serious consequences arising from pulmonary arterial aneurysms seen in BD and HSS. Also, the issue of anticoagulation in these patients is challenging and requires further deliberation.

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“…However, other clinical features are not specific since thrombophlebitis and aneurysm can occur anywhere, including in the hepatic artery, iliac artery, vena cava, cardiac chambers, femoral vein, iliac vein, and dural sinuses [2]. The patients may present cough, dyspnea, pulmonary hypertension, intracranial hypertension, fever, chills, chest pain, and hemoptysis [1,3].…”

Section: Discussionmentioning

confidence: 99%

“…Radiologic evaluations, including chest X-ray and chest computed tomography, could be useful only in few patients with large aneurysms. Conventional angiography is regarded as a gold standard of pulmonary artery aneurysms [2]. However, when the patient has thrombosis in the vena cava and has a high risk of aneurysmal rupture, angiography should not be performed.…”

Section: Discussionmentioning

confidence: 99%

Massive Hemoptysis Caused by Atypical Behcet's Disease

Lee

Hoon

Kim

2014

Korean J Thorac Cardiovasc Surg

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Pulmonary and/or bronchial aneurysm with systemic thrombophlebitis can be seen in the case of both Behcet's disease and Hughes-Stovin syndrome. These two diseases may be indistinguishable since the clinical, radiological, and histopathological findings are similar in both cases. In particular, from the perspective of pulmonary involvement, hemoptysis is the leading cause of death in both conditions and is followed by aneurysmal rupture. Here, we report a case in which surgical resection was performed for a patient presenting recurrent hemoptysis as an atypical presentation of Behcet's disease.

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Rare case of multiple pulmonary artery aneurysms with caval thrombosis — Hughes-Stovin syndrome

Cited by 18 publications

References 8 publications

A case of Hughes–Stovin syndrome (incomplete Behçet’s disease) with extensive arterial involvement

A case of Hughes–Stovin syndrome (incomplete Behçet’s disease) with extensive arterial involvement

Hughes-Stovin Syndrome

Massive Hemoptysis Caused by Atypical Behcet's Disease

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