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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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. More on this topic Pulmonary artery sarcoma mimicking pulmonary embolism: The formation of these emboli has been attributed to the inflammatory response of the endothelial cells lining the vessels [ 26 ].
The radiology of the Hughes-Stovin syndrome. Several proposed theories have attempted to explain the manifestations of this rare entity [ 1628 ]. Transcatheter arterial embolization For patients who are not suitable candidates for aggressive surgical intervention, transcatheter arterial embolization, being a less invasive procedure, offers a suitable and effective alternative in HSS [ 8 ].
The laboratory findings in HSS patients are non-specific.
Earlier diagnosis could be in some cases hugehs, allowing the prevention of the development of potentially life-threatening pulmonary artery aneurysms [ 9 ]. 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. Low pressure giant pulmonary artery aneurysms in the adult: The syndrome has not been referred to by any other synonym in medical literature.
Patients with HSS usually present with cough, dyspnea, fever, chest pain and haemoptysis. The documents contained in this web site are presented for information purposes only. Pulmonary manifestations of Behcet’s disease.
Bronchial syndroke hypertrophy secondary to ischemia that, in turn, has been attributed to the pulmonary artery occlusion [ 21 ]. However other clinical presentations can overlap significantly; both are more common in young men, the pulmonary manifestations of both can be identical, syndroke the histology of the aneurysms in both entities can be similar [ 8 ].
Immunosuppression has the potential to stabilize small aneurysms in the pulmonary vasculature [ 8 ], and in some cases can even make them regress [ 27 ].
Hughes–Stovin syndrome – Wikipedia
Although most of the hugghes 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. Radiologic findings syndrme the diagnosis of Hughes-Stovin syndrome. For pulmonary aneurysms, EULAR has recommended the continued use of cyclophosphamide for two years followed by azathioprine [ 58 ].
A growing body of evidence is suggestive of the active role of T-cell mediated immune mechanisms and responses in BD.
Retrieved from ” https: This article syndroome been cited by other articles in PMC. Table 2 Autoantibodies proposed to be involved in pathogenesis of Behcet’s disease adapted from Mendoza-Pinto et al [ 5 synvrome and Kapsimali et al [ 36 ]. The diagnosis of HSS can be difficult owing to a non-specific set of findings. Patients with thrombosis in the vena cava may have engorged abdominal superficial veins [ 26 ]. The pathogenesis of Hughes-Stovin syndrome is not known.
Infectious agents implicated in the pathogenesis of Behcet’s disease adapted from Mendoza-Pinto et al [ 5 ] and Kapsimali et al [ 36 synndrome. J Thorac Cardiovasc Surg.
HSS is a rare disorder of unknown etiology.
Multiple aneurysms of the pulmonary artery following recurrent septic pulmonary embolism Hughes-Stovin syndrome: These aspects have been highlighted for focused research in the future. Multiple pulmonary arterial aneurysms in Behcet’s disease and Hughes-Stovin syndrome.
Although the event can simply be attributed to the failure of the Greenfield filter, the possibility of the in situ formation of the pulmonary embolus as a separate entity from the deep venous thrombus can’t be ignored.
In this section, relevant EULAR recommendations with regards to the management of BD disease have been referred to, especially with regards to vascular disease. Journal List Cases J v.
Abstract Background Hughes-Stovin syndrome is a rare entity.