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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The exact etiology and pathogenesis of HSS is unknown; possible causes include infections and angiodysplasia.
Earlier diagnosis could be in some cases crucial, allowing the prevention of the development of potentially life-threatening pulmonary artery aneurysms [ 9 ]. Histologic studies show destruction of the arterial wall and perivascular lymphomonocytic infiltration of capillaries and venules.
His vital signs including temperature, arterial pressure and heart rate were normal. Summary and related texts. Tsai et al have recommended that in patients with pulmonary embolism and HSS, anticoagulation should be used very judiciously. Successful cyclophosphamide therapy with complete resolution of pulmonary artery aneurysm in Hughes-Stovin syndrome patient. However, surgical risks merit serious consideration and must be discussed with the patient.
Furthermore, Ketchum et al showed that 3D volume rendering analysis can detect morphologically abnormal, tortuous branches of bronchial arteries even before aneurysm formation [ 1249 ]. 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.
CT findings of pulmonary artery aneurysms during treatment for Behcet’s disease. Aneurysms usually involve the pulmonary arteries and the bronchial arteries resulting stogin subsequent haemoptysis. syndromf
The raised intracranial pressure also accounts for the papilledema of the optic disc observed in some patients with HSS [ 20 ]. However, immunologic mechanisms, different from those in other autoimmune diseases, are believed to be involved in the pathogenesis hugues BD. The radiology of the Hughes-Stovin syndrome. In general, there is a predisposition for thrombus formation affecting the peripheral veins.
Extensive microbiological investigation found no evidence of infection. Although most of the evidence put forward to refute the role of an infectious agent in the etiology and pathogenesis of HSS is based on negative blood and other body fluid cultures, more robust objective assessment sotvin needed through the use of electron microscopy or 16 sRNA studies.
Interplay of multiple factors such huges genetic, environmental, immunological and endothelial is most likely to be involved in the pathogenesis of BD [ 36 ]. Sign In or Create an Account.
These aneurysms generally involve the pulmonary and bronchial arteries but can also occur anywhere in systemic circulation. This article has been cited by other articles in PMC. Differential diagnosis The pulmonary manifestations of HSS and BD have been reported to be shovin, but the two syndromes can be synfrome on the basis of the absence of mucocutaneous findings in HSS.
UK performed the literature search, interpreted the data and drafted the manuscript. In fact, HSS and BD are the only vasculitides associated with the development of pulmonary artery aneurysms [ 44041 ]. Tissue pathology demonstrated necrotising lymphocytic vasculitis with pulmonary infarction. Chest x-ray demonstrated a right lower zone circumscribed lesion.
Orphanet: Hughes Stovin syndrome
Clinical examination was negative. Anticoagulants and thombolytic agents are generally considered contraindicated due to an increased risk of fatal hemorrhage, hugbes though they confer a beneficial effect in an embolic state [ 37 ].
An unusual case of Hughes-Stovin syndrome.
Cyclophosphamide, in particular, is a favored therapeutic agent in this regard. Similarities in pulmonary involvement between Behcet’s disease and HSS adapted from Erkan et al [ 38 ].
However, surgical risks merit serious consideration and must be discussed with the patient. Diagnostic criteria and differential diagnosis of Behcets disease. Contrast Enchanced Computed Tomography CT of the abdomen and pelvis was also performed in order to exclude a major intra-abdominal malignancy and thrombosis of left external iliac vein was depicted as well. Although most of the evidence put forward to refute the role of an infectious agent in the etiology and pathogenesis of HSS is based on negative blood and other body fluid cultures, more robust objective assessment is clearly needed through the use of electron microscopy or 16 sRNA studies.
Open in a separate window. The also performed the acquisition, analysis and interpretation of data. Received Nov 28; Accepted Apr Identification of aneurysms in the bronchial arteries should be treated by bronchial artery embolization [ 3 ].
Homocysteine causes thrombosis through multiple mechanisms including the activation of platelets, increased thrombin formation, impairment of fibrinolysis and endothelial dysfunction through lipid peroxidation and endothelial injury [ 45 ]. Open in a separate window. Antibiotics have no proven role in the management of HSS [ 122934 ].
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The syndrome has not been referred to by any other synonym in medical literature. Unusual case of cor pulmonale. Bronchoscopy Bronchoscopy is often done in HSS patients who present with hemoptysis. More on this topic Pulmonary artery sarcoma mimicking pulmonary embolism: He was treated with antibiotics for community-acquired pneumonia. However, early diagnosis and timely intervention is crucial in improving the prognosis. Future directions There is a lack of clear diagnostic criteria and management guidelines for HSS.
Diagnostic considerations The diagnosis of HSS can be difficult owing to a non-specific set of findings. J Med Case Reports.
Hughes-Stovin Syndrome: a case report and review of the literature
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. 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 ].
However, infectious agents, as an etiology for HSS, have generally received less attention due to two reasons.