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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However, it is only safe to perform this procedure in the early stages [ 72 ].
Written informed consent was obtained from the patient for publication of this case report and accompanying images. Future research should focus on the immunogenetic relationship between HSS and BD, optimization of immunosuppressive strategies through prospective controlled trials and evaluation of the long-term outcomes of embolization procedures.
Medical management includes the use of steroids and cytotoxic agents. Check this box if you atovin to receive a copy of your message. Bronchoscopy is often done in HSS patients who present with hemoptysis.
Discussion Hughes-Stovin syndrome is a rare clinical condition characterized by multiple pulmonary artery aneurysms and peripheral venous thrombosis, first described in [ 1 ].
Also, the issue of anticoagulation in these patients is challenging and requires further deliberation. A growing body of evidence is suggestive of the active role of T-cell mediated immune mechanisms and responses in BD. Arterial embolization is also an acceptable therapeutic option in patients with severe or recurrent hemoptysis [ 21 ]. A year-old male from Missouri, USA was admitted with haemoptysis following 1 month of intermittent fevers, non-productive cough and weight loss.
Hughes and Stovin’s original theory [ 1 ] suggested that degenerative bronchial arteries lead to changes in the vasa vasorum of the pulmonary arteries and the development of aneurysms. Patients, mostly men aged 12—40 years may present with haemoptysis, cough, dyspnea, chest pain, and signs of pulmonary hypertension. For permissions, please email: Conventional angiography can be used for better evaluation of the pulmonary aneurysms and assessment of angiodysplastic bronchial arteries in Hughes-Stovin syndrome.
Organized emboli of the tertiary pulmonary arteries. The elastic and muscular fibers become annihilated whereas tunica media is completely filled with lymphocytes, plasma cells and foam cells in the affected vessels. One other syndrome, Behcet’s disease BDis also associated with this aneurysm-thrombosis combination. Bronchial artery embolization in the management of hemoptysis: New approach in the diagnosis of and therapy for Hughes-Stovin syndrome.
Symptoms of thrombophlebitis b. 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.
Contrast-Enhanced MRA and Contrast-Enhanced MDCTA may provide an alternative [ 12 – 14 ] while 3D volume rendering analysis can ideally visualize the presence of an increased number of morphologically abnormal bronchial arteries even before aneurysm formation [ 9 ]. J Thorac Cardiovasc Surg.
Heat shock proteins – Hughds for heat shock proteins of mycobacteria and streptococci suggested in Behcet’s disease. Abstract Background Hughes-Stovin syndrome is a rare entity. The documents contained in this web site are presented for information purposes only. The exact etiology and pathogenesis of HSS is unknown; possible causes include infections and angiodysplasia.
Identification of aneurysms in the bronchial arteries should be treated by bronchial artery embolization [ 3 ]. Aneurysms of arterial origin portend a poorer prognostication than venular aneurysms [ 15 ].
Hughes-Stovin Syndrome: a case report and review of the literature
Segmental pulmonary artery aneurysms with peripheral venous thrombosis. Orphanet J Rare Dis. Magnetic resonance angiography Magnetic resonance angiography MRA is relatively less sensitive than helical CT for picking up small aneurysms [ synxrome264749 ].
Neutrophils exhibit increased generation of reactive oxygen species ROSphagocytic capacity and cytokine production as well [ 536 ]. Pulmonary artery aneurysms PAA are uncommon, with a diverse differential diagnosis.
Despite this, it should be noted stovinn the European League Against Rheumatism EULAR has acknowledged the need syndro,e properly designed and robust prospective studies for improving management strategies even for BD [ 5859 ]. Haemoptysis resolved with intravenous methylprednisolone treatment after about 25 days. However, a decade earlier, Adamantiades had reported the disease as well. Regardless the presence of deep venous hguhes, the pulmonary artery thrombi may originate in situ, secondary to the pulmonary artery wall inflammation.
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 synerome to be considered.
This is only in patients with embolisms in the main pulmonary artery that lead to life-threatening clinical deterioration and hemodynamic instability [ 26 ]. Despite the presence of thrombosis, anticoagulants are contraindicated due to the risk of life-threatening PAA rupture.
Magnetic resonance angiography MRA is relatively less sensitive than helical CT for picking up small aneurysms [ 20264749 ]. Furthermore, Ketchum et al showed that 3D volume rendering analysis can detect morphologically abnormal, tortuous branches of bronchial arteries even before aneurysm formation [ 1249 ].
You can help Wikipedia by expanding it. Multiple pulmonary aneurysms secondary to Hughes-Stovin syndrome: Bridge to immunogenetics Classically hugjes most convincingly, the association of BD has been described with the human leukocyte antigen HLA B51 in literature. For the 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 to remove the aneurysms based on the data from published case reports.
J Med Case Reports. Several proposed theories have attempted to explain the manifestations of this rare entity [ 1628 ]. Multiple, small PAA warrant early immunosuppression. Surgical resection syndome the affected segments of the lung is to be considered in cases of high risk rupture aneurysms limited to one segment or one lung [ 7 ]. Past medical history was significant for recurrent oral ulcers. The pre-operative use of doxycycline has been recommended because of its potential role in off-setting these deleterious post-operative events [ 69 ].