Hughes–Stovin syndrome is a rare autoimmune disorder of unknown cause that is characterized by the combination of multiple pulmonary artery aneurysms and . Learning points for clinicians. Pulmonary artery aneurysms (PAA) have a diverse differential diagnosis. Vasculitic features, without evidence of. Hughes-Stovin syndrome (HSS) is a rare autoimmune disorder, characterized by deep venous thrombosis (DVT) and pulmonary and/or bronchial aneurysms.
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Prevalence is unknown but fewer than 30 cases have been reported in the literature since its first description in by Hughes and Stovin. Receive exclusive offers and updates from Oxford Academic. Diagnostic considerations The diagnosis of HSS can be difficult owing to a non-specific set of findings. However, surgical risks merit serious consideration and must be discussed with the patient. Contrast Enchanced Computed Tomography CT of the abdomen and pelvis was also performed in order to exclude a major intra-abdominal malignancy and thrombosis of left external iliac vein was depicted as well.
Bronchoscopy is often done in HSS patients who present with hemoptysis. However, early diagnosis and timely intervention is crucial in improving the prognosis. Prognosis As most patients with HSS are diagnosed late in the disease course, the syndrome is associated with significant mortality due to massive hemoptysis resulting from PAA rupture or systemic bronchial artery hypertrophy secondary to ischemia related to the pulmonary artery occlusion. Thrombophilia is not believed to be a major contributory factor to the development of thrombosis in these patients [ 15 ].
Severe hemoptysis of pulmonary arterial origin: Several proposed theories have attempted to explain the manifestations of this rare entity [ 1628 ]. For hugyes 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.
Hughes–Stovin syndrome – Wikipedia
Treatment of venous thrombosis associated with Behcet’s disease: Anticoagulants and thrombolytic agents Anticoagulants and thombolytic agents are generally considered contraindicated due to an increased risk of fatal hemorrhage, even though they confer a beneficial effect in an embolic state [ 37 ].
J Vasc Interv Radiol. According to the initial hypothesis [ 1 ], pulmonary artery aneurysms may develop from a degenerative defect in the bronchial arteries or may even be mycotic in origin resulting from emboli infected with low-grade virulence organisms.
However systemic bronchial artery hypertrophy secondary to ischemia related to the pulmonary artery occlusion could also be the origin shovin bleeding [ 310 ]. Unless these arenas are elucidated, the “pathogenic kinship” between the two conditions remains obscure [ 18 ] and should be examined with scientific skepticism hughess perspicacity.
Mahlo et al [ 17 ] and Herb et al [ 21 ] recorded distorted and dilated bronchial arteries with convoluted small branches when hugnes performed digital subtraction angiography of the bronchial arteries. J Med Assoc Thai. These aspects have been highlighted for focused research in the future. Behcet’s syndrome with hemoptysis and pulmonary lesions.
Clinical description Patients mostly men aged years generally present with the nonspecific signs of PAA hemoptysis, cough, dyspnea, chest pain, and signs of pulmonary hypertensionfollowing a history of peripheral venous thrombosis. Hughes and Stovin’s original theory [ 1 ] suggested that degenerative bronchial arteries lead to changes in the vasa vasorum of the pulmonary arteries and the development of aneurysms.
After 10 days on heparin stovim, dry cough begun, followed by an hughess of moderate haemoptysis — ml.
Sign In or Create an Account. However, whether the two conditions are identical is open to both debate and speculation as the exact pathophysiology of both syndromes remains unclear.
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Hughes and Stovin suggested that the structural changes in the bronchial arteries impaired the provision of adequate nutrition to the pulmonary arteries through the vasa vasorum. Hughes-Stovin syndrome is a rare entity. This is only in patients with embolisms in the main pulmonary artery that lead to life-threatening clinical deterioration and hemodynamic instability [ 26 ].
Aneurysmal rupture; it is the leading cause of death in patients of HSS. Successful cyclophosphamide therapy with complete resolution of pulmonary artery aneurysm in Hughes-Stovin syndrome patient.
Hughes-Stovin Syndrome: a case report and review of the literature
All authors have read and approved the final manuscript. Ophthalmological examination bughes uveitis, vitritis or retinal vasculitis and he did not have genital ulceration or skin manifestations of BD.
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Identification of aneurysms in the bronchial arteries should be treated by bronchial artery embolization [ 3 ]. D ICD – Journal List Cases J v.
It is characterized by the association of multiple pulmonary artery aneurysms PAAs and peripheral venous thrombosis. Citing articles via Google Scholar. Trauma for example, from a Swan-Ganz catheter. Management Owing to the lack of controlled trials, there are no standard treatment guidelines for the management of HSS. Table 3 Causes for pulmonary artery aneurysms without arteriovenous communication adapted from Fischer et al [ 18 ].
PAA resection by thoracotomy and lower lobe basal segmentectomy was performed. The etiology of HSS is unknown; however, it is assumed that HSS is a form of vasculitis following a similar mechanism of pathogenesis to that thought to etovin involved in BD. The documents contained in this web site are presented for information purposes only.