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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Multiple pulmonary artery aneurysms and xyndrome venous thromboses: Identification of aneurysms in the bronchial arteries should be treated by bronchial artery embolization [ 3 ]. 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.

Treatment strategies for Behcet’s disease. In fact, multi-detector row helical CT angiography now offers more precise visualization of large systemic arteries than does conventional angiography. Scleromalacia perforans in rheumatoid arthritis. UK performed the literature search, interpreted the data and drafted the manuscript. Interestingly, in support of the infectious etiology of BD, it synndrome been syndroms that proximity to the Silk Road and interactive trade activities may have provided the necessary bridge for the extension of the disease between the Mediterranean and hughess East [ 5 ].

Check this box if you wish to receive a copy of your message. Epidemiology HSS is an exceedingly rare disorder with less than 40 published cases in English medical literature. There is a need to clearly elucidate the genetic, etiologic and pathologic basis for HSS in the future.

Less than 40 published cases of HSS have been described in English medical literature so far. Chest x-ray demonstrated a right lower zone circumscribed lesion.


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 ].

There are also studies which have shown a female preponderance [ 5253 ]. Recurrent phlebitis also commonly involves the large vessels, resulting in thrombus formation.

Surgical resection provides an effective treatment option for patients with unilateral or localized PAAs snydrome less invasive approaches such as transcatheter embolization stovn be feasible in some cases.

An unusual case of Hughes-Stovin syndrome. Patients with thrombosis in the vena cava may have engorged abdominal superficial veins [ 26 ]. However, a decade earlier, Adamantiades had reported the disease as well. Similarly Mahlo et al. Am J Emerg Med. Aneurysmal rupture leading to massive hemoptysis and death These stages usually evolve successively. However, immunologic mechanisms, different from those in other autoimmune diseases, are believed sotvin be involved in the pathogenesis of BD.

Orphanet: Hughes Stovin syndrome

In support of this theory, pulmonary aneurysms have been preceded by infections in some cases, including scrotal abscesses [ 129 ], epididymitis [ 2 ] and oophoritis [ 32 ]. Received Dec 15; Accepted Jan However, early diagnosis and timely intervention is crucial in improving the prognosis.

Hughes—Stovin syndrome is a rare autoimmune disorder of unknown cause that is characterized by the combination of multiple pulmonary artery aneurysms and deep vein thrombosis. Comparison with Conventional Angiography. 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 ].

Br J Dis Chest.

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 ]. Unless these arenas are elucidated, the “pathogenic kinship” between the two conditions remains obscure [ 18 ] and should stoviin examined with scientific skepticism and perspicacity.


Clinical description Patients mostly men aged huughes generally present with the nonspecific signs of PAA hemoptysis, cough, dyspnea, chest pain, and sgndrome of pulmonary hypertensionfollowing a history of peripheral venous thrombosis. The syndrome is most often treated by immunosuppressant, either systemic corticosteroids or cytotoxic agents a combination of cyclophosphamide and glucocorticoidsin patients without or with small amount of hemoptysis in order to stabilize the pulmonary artery aneurysms [ 2 ], or even make them disappear [ 15 ].

Hughes–Stovin syndrome

Though most patients with Hughes-Stovin syndrome will present atovin 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 exact etiology and pathogenesis of HSS is unknown; possible causes include infections and angiodysplasia. Radiologic stoovin in the diagnosis of Hughes-Stovin syndrome.

For pulmonary aneurysms, EULAR has recommended the continued use of cyclophosphamide for two years followed by azathioprine [ 58 ]. However, Durieux et al [ 37 ] described a dense neutrophilic infiltrate in the walls of the vessels in HSS.

Strictly speaking, BD is not considered an autoimmune disease [ 36 ] because of the following reasons: Arch Pathol Chic ;