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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For the clinical diagnosis of BD, the patient must have recurrent oral ulceration with atleast two of the following clinical manifestations: It also aids in assessment of angiodysplastic bronchial arteries in HSS.
Hughes–Stovin syndrome – Wikipedia
Extrapolation of the pathologic model for Behcet’s disease As mentioned earlier, although the exact pathologic basis for HSS is unclear, it may be similar to the model for BD as the two conditions share many features. CT scan of the chest showing ill defined infiltrate in lower lobe of the right lung. Etiopathogenesis of Behcet’s disease.
Unusual case of cor pulmonale. Finally, the development of better therapeutic agents is needed to address and prevent the serious consequences arising from pulmonary arterial aneurysms seen in BD and HSS. Aneurysmal rupture; it is the leading cause stkvin death in patients of HSS.
Surgical management For the 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 to remove the aneurysms based on the data from published case reports.
Mahlo et al [ 17 ] and Herb et al [ 21 ] recorded distorted and dilated bronchial hughse with convoluted small branches when they performed digital subtraction angiography of the bronchial arteries. Similarly, the pathogenesis of pulmonary artery aneurysms in Hughes-Stovin syndrome has been attributed to weakening of the vessel wall due to inflammation, which corresponds well to the observation by Ketchum et al, that the aneurysms develop at the locations of prior thrombus and abnormal enhancement [ 9 ].
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Recurrent phlebitis also commonly involves the large vessels, resulting in thrombus hugghes. Authors have performed embolization with several agents including steel coils, ethibloc and an epoxy, isobutyl cyanoacrylate [ 8 ]. More on this topic Pulmonary artery sarcoma mimicking pulmonary embolism: This article has been cited by other articles in PMC. In solitary peripheral PAA, we recommend operation or embolization for rapidly growing or high-pressure aneurysms.
Anti-HSV-1 antibodies observed more commonly in patients with Behcet’s disease than controls.
However, a decade earlier, Adamantiades had reported the disease as well. Diagnostic criteria and differential diagnosis of Behcets disease. We are greatly indebted to the reviewers for their valuable suggestions and critique.
Curr Opin Pulm Med.
National Center for Biotechnology InformationU. Prevalence is unknown but fewer than 30 cases have been reported in the literature since its first description in by Hughes and Stovin.
Regardless the presence of deep venous thrombosis, the pulmonary artery thrombi may originate in situ, secondary to the pulmonary artery wall inflammation. Specialised Social Services Eurordis directory. 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 ].
In turn, these events led to inflammation, damage to the elastic tissue and creation of arterial aneurysms [ 21 ]. Although most are asymptomatic at diagnosis, fatal aneurysm rupture syndromw in one-third and dissection in one-fifth. Venous thrombosis can also be considered to arise as a consequence of septic emboli, bacterial toxins or huvhes reactions [ 33 ].
CT angiography of pulmonary artery aneurysms in Hughes-Stovin syndrome. Angiodysplasia Angiodysplasia of bronchial arteries is another debatable hypothesis to account for the vascular changes [ 162837 ].
Hughes-Stovin Syndrome: a case report and review of the literature
Bronchoscopy is often done in HSS patients who present with hemoptysis. Br J Dis Chest. The exact etiology and pathogenesis of HSS is unknown; possible causes include infections and angiodysplasia.