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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Stoin are greatly indebted to the reviewers for their valuable suggestions and critique. Etiology The etiology of HSS is unknown; however, it is assumed that HSS is a form of vasculitis following a similar mechanism of pathogenesis to that thought to be involved in BD. Table 3 Causes for pulmonary artery aneurysms without arteriovenous communication adapted from Fischer et al [ 18 ].

Although it should be acknowledged that HSS and BD share many clinical, radiological and histological features, the suggestion that they are, in essence, the same disease can neither be currently accepted nor categorically refuted because the pathologic, genetic and etiologic basis of both conditions has not been clearly unraveled.

However systemic bronchial artery hypertrophy secondary to ischemia related to the etovin artery occlusion could also be the origin of bleeding [ 310 ]. Hemoptysis caused by Hughes-Stovin syndrome. Pathogenesis, imaging investigation and treatment of this syndrome are also briefly discussed. His vital signs including temperature, arterial pressure and heart rate were normal.

Signs and symptoms of Hughes-Stovin Syndrome.

Unusual right ventricular thrombus in a woman with Hughes-Stovin syndrome. Along with the initiation of medical management, an urgent consultation should be given to the cardiothoracic surgery team, especially if the patient presents with severe or recalcitrant hemoptysis [ 26 ].


Other associated features reported in Hughes-Stovin syndrome include fever and elevated intracranial hypertension Since its first description, there have been only a few reports of its occurrence in the English medical literature [ 2 ]. For pulmonary aneurysms, EULAR has recommended the continued use of cyclophosphamide for two years followed by azathioprine [ 58 ].

Some authors have suggested that HSS may actually be a partially manifested BD owing to their similar findings instead of a novel syndrome [ 16193839 ]. An 18 years old, Greek male patient presented with edema of his left leg.

In general, there is a predisposition for thrombus formation affecting the peripheral veins. This places physicians on the horns of a dilemma because of the pro-thrombotic nature of the syndrome and the occurrence of potentially life-threatening events such as intracardiac thrombi or pulmonary embolism.

Etiology and pathogenesis The exact etiology and pathogenesis of HSS is currently unknown. Published online Jan There is a lack of clear diagnostic criteria and management guidelines for HSS. This is only in patients with embolisms wyndrome the main pulmonary artery that lead to life-threatening clinical deterioration and hemodynamic instability [ 26 ].

Received Dec 15; Accepted Jan This page was last edited on 9 Julyat Anticoagulation is typically contraindicated by bleeding risk. The natural course of the illness is usually fatal because of fulminant haemoptysis.

Hughes-Stovin Syndrome: a case report and review of the literature

Diagnosis of HSS is made on the basis of the clinical picture association of venous thrombosis and PAAs in a young patientpatient history and imaging studies chest radiographs, conventional angiography or helical computed tomography for detection and evaluation of the PAAs.


However, infectious agents, as an etiology for HSS, have generally received less attention due to two reasons.

Transcatheter arterial embolization has emerged as a less invasive alternative to surgery in selected cases of HSS. J Vasc Interv Radiol.

The perivascular infiltrate extends into the adventia and into the overlying thrombus. We report the case of an 18 years old, greek male patient with Hughes-Stovin syndrome, who initially presented with deep vein thrombosis. There is also a need to better elucidate the genetic basis and familial preponderance, if any, of HSS.

Infobox medical condition new Pages using infobox medical condition with unknown parameters Articles to be expanded from September All articles to be expanded Articles with empty sections from September All articles with empty sections Articles using small message boxes Hughee stub articles. Successful cyclophosphamide therapy with complete resolution of pulmonary artery aneurysm in Hughes-Stovin syndrome patient. Diagnosis and Discussion of Case 1. Patients with HSS usually present with cough, dyspnea, fever, chest pain and haemoptysis.

Hughes-Stovin Syndrome

Histological diagnosis Stovjn histopathologic findings of HSS [ 12 ] include diffuse dilatation and partial occlusion of the aneurysmal arteries, perivascular infiltration that is predominantly lympho-monocytic and diffuse proliferative sclerosis. He was treated with antibiotics for community-acquired pneumonia.

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