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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This section is empty. Competing interests The authors declare that they have no competing interests. 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 ]. Even enlargement of bronchial arteries when recognized, should be treated [ 11 ].
Successful cyclophosphamide therapy with complete resolution of pulmonary artery aneurysm in Hughes-Stovin syndrome patient.
His vital signs including temperature, arterial pressure and heart rate were normal. Chest Stoivn Enhanced-MSCT angiography axial section showing a partially thrombosed aneurysm of the right lower lobe pulmonary stovkn, and thrombosed segmental branch of the left lower lobe pulmonary artery. The development of better therapeutic agents is also needed to address and prevent the serious consequences arising from pulmonary arterial aneurysms seen in BD and HSS.
Am J Emerg Med. Hughes-Stovin syndrome with pulmonary angiitis and focal glomerulonephritis: It is believed that the aneurysms seen in BD arise either due to the process of obliterative endarteritis of the vasa vasorum or they are pseudo-aneurysms characterized by edematous vessel walls.
Strictly speaking, BD is not considered an autoimmune disease [ 36 ] because of the following reasons: Am J Emerg Med. Another point to consider is the possible role of deep venous thrombi in aneurysm formation. Prognosis Aneurysms of arterial origin portend a poorer prognostication than venular aneurysms [ 15 ].
Prevalence is unknown but fewer than 30 cases have been reported in the literature since its first description in by Hughes and Stovin. National Center for Biotechnology InformationU. Only comments written in English can hughs processed.
Cyclophosphamide, in particular, is a favored therapeutic agent in this regard. Multiple pulmonary artery aneurysms.
In comparison, the histology syndroem vascular lesions in BD is characterized by a triad of “neutrophilic infiltration”, “endothelial cell swelling” and “fibrinoid necrosis” [ 5 ]. Secondly, there has been a lack of positive blood cultures in the evaluation of patients with HSS [ 1 – 3112931323435 ].
For example, Herb et al have reported HSS in a patient who had an aneurysm of the left hepatic artery [ 21 ].
Past medical history was significant for recurrent oral ulcers. Also, the issue of anticoagulation in these patients is challenging and requires further deliberation. Clinical description 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.
However, whether stivin two conditions are identical is open to both debate and speculation as sndrome exact pathophysiology of both syndromes remains unclear.
There are also studies which have shown a female preponderance [ 5253 ]. Eur J Cardiothorac Surg. 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. 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.
In general, there is a predisposition for thrombus formation affecting the peripheral veins. HSS does not appear to have preponderance for any geographic location.
However, their use may be considered with great care under special circumstances, for instance, intracardiac thrombi or massive pulmonary embolism. Pulmonary Disease in Behcet’s syndrome.
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.
Br J Dis Chest. Most of the data on HSS is in the form of sporadic case reports. Table 2 Autoantibodies proposed to be involved in pathogenesis of Behcet’s disease adapted from Mendoza-Pinto et al [ 5 ] and Kapsimali et al [ 36 ].
Orphanet: Hughes Stovin syndrome
The exact etiology and pathogenesis of HSS is currently unknown. In turn, these events led to inflammation, damage to the elastic tissue and creation of arterial aneurysms [ 21 ]. A growing body of evidence is suggestive of the active role of T-cell mediated immune mechanisms and responses in BD. Learning points for clinicians. N Engl J Med.
New approach in the diagnosis of etovin therapy for Hughes-Stovin syndrome. The development of better therapeutic agents is also needed to address and prevent the serious consequences arising from pulmonary arterial aneurysms seen in BD and HSS.
In general there is a thrombus formation predisposition affecting the peripheral veins. In fact, HSS and BD are the only vasculitides associated with the development of pulmonary artery aneurysms [ hugehs4041 ].