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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Histologic studies show destruction of the arterial wall and syndrone lymphomonocytic infiltration of capillaries and venules [ 5 ]. Case presentation An 18 years old, Greek male patient presented with edema of his left leg. Citing articles via Google Scholar.
Regardless the presence of deep venous thrombosis, the pulmonary artery thrombi may originate in situ, secondary to the pulmonary artery wall inflammation. For permissions, please email: Differential diagnosis The pulmonary manifestations of HSS and BD have been reported to be identical, but the two syndromes can be distinguished on the basis of the absence of mucocutaneous findings in HSS.
Trauma for example, from a Swan-Ganz catheter. We report the case of an 18 years old, greek male patient with Hughes-Stovin syndrome, who initially presented with deep vein thrombosis.
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.
Finally, haemoptysis could be attributed secondary to both pre-mentioned mechanisms [ 11 ]. 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 synrrome vasculitis.
Physical examination found fever, tachycardia and reduced air entry to his right lung base.
The volume-rendered CT images according to Ketchum et al show prominent and sydnrome bronchial artery branches that apparently supply a web of smaller vessels at the sites of pulmonary artery wall inflammation even before the actual aneurysm formation [ 9 ].
Multiple pulmonary aneurysms secondary to Hughes-Stovin syndrome: Arterial embolization is also an acceptable therapeutic option in patients with severe or recurrent hemoptysis [ 21 ]. Rarely, the patients may require repeat embolization because the arterial lesions may become recanalized or revascularized [ 21 ].
It is speculated that HLA-B51 forms an integral part of an immunologic axis in patients with BD that interacts with cross-reacting self-antigens and immunoglobulin-like receptors on immune cells of the body [ 36 ]. Although most are asymptomatic at diagnosis, fatal aneurysm rupture occurs in one-third and dissection in one-fifth.
Causes for pulmonary artery aneurysms without arteriovenous communication adapted from Fischer et al [ 18 ]. Other agents that have syndrmoe variably used in the treatment of HSS include colcichine, cyclosporine and azathioprine [ 81538 ].
Aneurysmal rupture; it is the leading cause of death in patients of HSS.
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 ]. You can help by adding to it. Arch Pathol Chic ; Syndrome of pulmonary artery aneurysms, pulmonary artery thrombi and peripheral venous thrombi. This article has been cited by other articles in PMC. The natural course of the illness is usually fatal because of fulminant haemoptysis.
EULAR recommends the use of steroids, azathioprine, cyclophosphamide or cyclosporine A for the management of acute deep vein thrombosis of BD while cyclophosphamide and corticosteroids have been recommended for pulmonary and peripheral arterial aneurysms in BD [ 58 ]. Management and treatment Initial management of HSS often involves administration of corticosteroids, usually in combination with cytotoxic agents intravenous cyclophosphamide followed by oral azathioprine to stabilize the PAAs.
There is also a need to better elucidate the genetic basis and familial preponderance, if any, of HSS. Management Owing to the lack of controlled trials, there are no standard treatment guidelines for the management of HSS.
Hughes–Stovin syndrome – Wikipedia
Immunological testing found a weak-positive ANA 1: Author information Article notes Copyright and License information Disclaimer. We hughfs greatly indebted to the reviewers for their valuable suggestions and critique.
Antibiotics have no proven role in HSS while anticoagulants and thombolytic agents are generally contraindicated due to an increased risk of fatal hemorrhage.
Septic embolisms and abscesses have been proposed as the cause of pulmonary aneurysms by some authorities [ 329 – 31 ].
Venous thrombosis can also be considered to arise as a consequence of septic emboli, bacterial toxins or hyperergic reactions [ 33 ]. Author information Article notes Copyright and License information Disclaimer.
However, surgical risks merit serious consideration and must be discussed with the patient. Patients with HSS can develop pulmonary embolism.