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 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. However, it cannot be performed in all patients with venous thromboses. This is only in patients with embolisms in the main pulmonary artery that lead to life-threatening clinical deterioration and hemodynamic instability [ 26 ].
Summary and related texts. CT angiography of pulmonary artery aneurysms in Hughes-Stovin syndrome. Unclear role, distribution and pathogenetic relationship gughes ASCA antibodies in patients with Behcet’s disease. In addition, neutrophil hyperactivation, a key component of inflammatory vasculitis seen in BD, occurs secondary to the release of the battery of cytokines from the APCs and T-cells.
Helical computed tomography However, it may not be possible to perform angiography in all patients; especially in patients with thromboses in the vena cava which limit the passage of the catheter [ 26 ].
Patients presenting with severe hemoptysis may require hughea of mechanical ventilator support [ 65 ]. At review his vital signs were normal and his platelet count and coagulation tests were within normal limits. Recurrent phlebitis frequently involves the large vessels resulting in thromboembolism, with even reports of thrombosis of the vena cava, cardiac chambers, jugular vein, iliac vein, femoral vein and dural sinuses [ 810161924 – 27 ].
The stoin infiltrate extends into stocin adventia and into the overlying thrombus.
UK performed the literature search, interpreted the data and drafted the manuscript. However, immunologic mechanisms, different from those in other autoimmune diseases, are believed to be involved in the pathogenesis of BD.
The exact etiology and pathogenesis of HSS is unknown; possible causes include infections and angiodysplasia. 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.
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However, Durieux et al [ 37 ] described a dense neutrophilic infiltrate in the walls of the vessels in HSS. Sign In or Create an Account. Unusual right ventricular thrombus in a woman with Hughes-Stovin syndrome.
Behcet’s syndrome with hemoptysis and pulmonary lesions. Symptoms of thrombophlebitis b.
Hughes-Stovin Syndrome: a case report and review of the literature
Macrophages containing hemosiderin may also be observed. Echocardiogram excluded pulmonary artery hypertension PAH and structural cardiac disease.
The characteristic picture seen is aneurysmal formation proximal to the occluded segments while distal to the interruption, signs of hypoperfusion are observed [ 48 ]. Histologic studies show destruction of the arterial wall and perivascular lymphomonocytic infiltration of capillaries and venules [ 5 ]. A timely diagnosis and intervention is imperative to prevent potentially life-threatening massive hemorrhage due to pulmonary aneurysms [ 12 ].
On the other hand, Meireles et al [ 35 ] reported the histologic findings of HSS in a patient’s necropsy as medial hypertrophy, intimal fibrosis with marked eosinophilic infiltrates. Chlamydia pneumoniae – IgG seropositivity for C. However, surgical risks merit serious consideration and must be discussed with the patient. Report of two cases and review of snydrome literature. Septic embolisms and abscesses have been proposed as the cause of pulmonary aneurysms by some authorities [ 329 – 31 ].
Though most patients with Hughes-Stovin syndrome will present with haemoptysis and advanced disease, dysplastic bronchial arteries and pathologic enhancement of pulmonary thromboemboli seen on CT pulmonary angiograms should alert the radiologist and raise suspicion of pulmonary vasculitis.
The pathogenesis of Hughes-Stovin syndrome is not known. Immunosuppression has the potential to stabilize small aneurysms in the pulmonary vasculature [ 8 ], and in some cases can even make them regress [ 27 ]. Conversely, it has been huyhes that the occlusion of the pulmonary arteries causes increased flow and pressure in the bronchial arteries which predisposes to the formation of bronchial artery aneurysms [ 21 ]. Conclusion In young men presenting with venous thrombosis as revealed on imaging examination, with platelet count and coagulation tests within normal and hemoptysis the eventuality of Hughes-Stovin syndrome is to be considered.
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. As such, it can be regarded as an emerging and effective standard in the diagnosis of pulmonary artery aneurysms because of its non-invasive nature, ease of performance stpvin increasing availability. 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 uhghes hypertrophy secondary to ischemia related to the pulmonary artery occlusion.
These aneurysms generally involve the pulmonary and bronchial arteries but can also occur anywhere in systemic circulation. Diagnosis and Discussion hugnes Case 1. He returned to America post-operatively for immunosuppressive treatment.
Also, the thrombin in the lower extremities is tightly adherent to the inflamed veins in BD and HSS patients [ 121937 ].