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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Haemoptysis resolved with intravenous methylprednisolone treatment after about 25 days. More on this topic Pulmonary artery sarcoma mimicking pulmonary embolism: 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.
Unless these arenas are elucidated, the “pathogenic kinship” between the two conditions remains obscure [ 18 ] and should be examined with scientific skepticism and perspicacity.
The steroids are usually administered as pulse IV therapy huhes by oral steroids usually with subsequent taper [ 15193845 ]. J Med Case Reports. Interestingly, in support of the infectious etiology of BD, it has hugjes suggested 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 the East [ 5 ].
Orphanet: Hughes Stovin syndrome
Past medical history was significant for recurrent oral ulcers. Infectious agents implicated in the pathogenesis of Behcet’s disease adapted from Mendoza-Pinto et al [ 5 ] and Kapsimali et al [ 36 ].
Strictly speaking, BD is not considered an autoimmune disease [ 36 ] because of the following reasons: Medical management includes the use of steroids and cytotoxic agents. Competing interests The authors declare that they have no competing interests.
BD primarily affects young adults, especially males [ 404151 ]. Histological diagnosis Classic 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. Heat shock proteins – Role for heat shock huhges of mycobacteria and streptococci suggested in Behcet’s disease. 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 ].
Overall, patients with HSS have a poor prognosis and aneurysmal stoin is the leading cause of death. Angiodysplasia Angiodysplasia of bronchial arteries is another debatable hypothesis to account for the vascular changes [ 162837 ].
Additional information Further information on this disease Classification s 2 Gene s 0 Clinical signs and symptoms Other website s 1. Arterial embolization is also an acceptable therapeutic option in patients with severe or recurrent hemoptysis [ 21 ]. This is how HSS has uhghes diagnosed in the majority of the case reports in literature. Aneurysmal rupture; it is the leading cause of death in patients of HSS.
Trauma for example, from a Swan-Ganz catheter 6. Report of two cases and review of the literature.
The characteristic picture seen is aneurysmal formation proximal to the occluded segments while distal to the interruption, signs of hypoperfusion are observed [ 48 ].
Antineutrophilic cytoplasmic antibody ANCA 3.
Interplay of multiple factors such as genetic, environmental, immunological and endothelial is most likely to be involved in the pathogenesis of BD [ 36 ]. Aneurysms of the pulmonary arteries. Segmental pulmonary artery aneurysms with peripheral venous thrombosis. For BD, Alexoudi et al have recommended that surgery be considered as a treatment of choice for vascular involvement in the following circumstances: In fact it is reported that multi-detector row helical CT angiography provides more precise depiction of bronchial and nonbronchial systemic arteries than does conventional angiography [ 14 ].
Streptococcus sanguis, Streptococcus mitis and Streptococcus salivarius – Attenuation of skin and arthritic involvement in Behcet’s disease after antibiotic administration.
Prevalence is unknown but fewer than 30 cases have been reported in the literature since its first description in by Hughes and Stovin. Patients with Huughes usually present with cough, dyspnea, fever, chest pain and haemoptysis. Typical symptoms are recurrent ssyndrome, chills, haemoptysis and coughs and it usually affects young men.
Based on clinical and histopathological findings, without meeting BD criteria, he was diagnosed with HSS. Finally, haemoptysis could be attributed secondary to both syndromee mechanisms [ 11 ]. Hughes-Stovin syndrome with pulmonary angiitis and focal glomerulonephritis: BD has been known to occur more commonly in geographic areas that fall between latitudes 30 and 45 degrees north [ 7 ]. There are also studies which have shown a female preponderance [ 5253 ].
Large pulmonary artery aneurysm rupture in Hughes-Stovin syndrome: Secondly, there has been a lack of positive blood cultures in the evaluation of patients with HSS [ 1 – 3112931323435 ].
Although BD is found all over the world, certain regions like Far East, the Mediterranean the ancient “Silk Road” and the Middle East have reported higher rates of prevalence [ 3640415154 ].
Related articles in PubMed Characterization of healthy hughse nonmelanoma-induced mouse utilizing the Stokes-Mueller decomposition. Anticoagulants and thrombolytic agents 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 ].
Etiopathogenesis of Behcet’s disease.
Bronchoscopy Bronchoscopy is often done in HSS patients who present with hemoptysis. Comparison with Conventional Angiography. The management of HSS can either be medical or surgical. 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 needed through the huhhes of electron microscopy or 16 sRNA studies. Some authors have suggested that HSS may actually be a partially manifested BD owing to their similar findings instead of a novel syndrome [ 16193839 ].