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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We report the case of an 18 years old, stocin male patient with Hughes-Stovin syndrome, who initially presented with deep vein thrombosis. 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 ].
MR brain venography showed chronic thrombosis of superior sagittal and transverse sinuses Fig. Antiplatelet agents In the absence of extensive thrombi, hguhes authors have suggested the use of antiplatelet agents such as low dose aspirin in patients [ 6364 ].
For permissions, please email: Pulmonary involvement in Behcet disease. Pulmonary arterial thrombo-embolism and pulmonary arterial mycotic aneurysms in cattle with vena caval thrombosis: On initial CT images a rim of enhancement at the huhhes defects may be depicted; this finding is consistent with inflammation — a finding that is not usually seen in acute or chronic pulmonary embolism [ 9 ].
Hughes-Stovin Syndrome: a case report and review of the literature
Definition and diagnostic criteria HSS is a rare disorder of unknown hugjes. The syndrome has not been referred to by any other synonym in medical literature. The perivascular infiltrate extends into the adventia and into the overlying thrombus.
However, the caveat here is that patients may still develop thrombosis despite adequate anticoagulation [ 38 ]. Antineutrophilic cytoplasmic antibody ANCA 3.
Hughes–Stovin syndrome – Wikipedia
The latter are usually formed after perforation [ 38 ]. Recurrent phlebitis also commonly involves the large vessels, resulting in thrombus formation. Depending upon the clinical response, steroids can be discontinued but cyclophosphamide is usually given for at least one year after complete remission [ 4 ]. Helicobacter pylori Almost equal proportion of patients with Behcet’s disease and controls had H.
Tissue pathology demonstrated necrotising lymphocytic vasculitis with pulmonary sgndrome. Pulmonary Disease in Behcet’s syndrome. Parvovirus B19 IgG antibodies reported more in patients with Behcet’s disease as compared tsovin controls. After 10 days on heparin therapy, dry cough begun, followed by an episode of moderate haemoptysis — ml.
However, early diagnosis and timely intervention is crucial in improving the prognosis. Pathogenesis, imaging investigation and treatment of this syndrome are also briefly discussed. Aneurysmal rupture leading to massive hemoptysis and death These stages usually evolve successively. If the site develops an erythematous papule after 48 hours, the test is positive [ 5 ]. Medical management includes the use of steroids and cytotoxic agents.
Physical examination found fever, tachycardia and reduced air entry to his right lung base. 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. However, it should be noted that selective pulmonary angiography can be hazardous as it carries the risk of aneurysm rupture [ 17 ].
Early diagnosis and timely intervention is, therefore, crucial in improving the prognosis of patients with HSS. 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 ].
Doppler ultrasound of extremities Peripheral venous thrombosis is an important part of HSS. However, their use may be considered with great care under special circumstances, for instance, intracardiac thrombi or massive pulmonary embolism. Cyclophosphamide, in particular, is a favored therapeutic agent in this regard.
British Journal of Diseases of the Chest. In the pulmonary veins, fibrosis and muscular medial thickening is seen. Most commonly, immunosuppressive therapy involving a combination regimen of glucocorticoids and cyclophosphamide has been employed as a first line medical syndroke in the treatment of HSS, although its effectiveness remains to be fully established [ 840 ].
Open in a separate window. For this reason, its population-based incidence can’t be determined.
Additional information Further information on this disease Classification s 2 Gene s 0 Clinical signs and symptoms Other website s 1. This article about a disease of the blood or immune system is huyhes stub. Helical computed tomography However, it may not be possible to perform angiography in synsrome patients; especially in patients with thromboses in the vena cava which limit the passage of the catheter [ 26 ].
Clinical examination was negative. Radiologic findings in the diagnosis of Hughes-Stovin syndrome.
The first and second phases huhhes to be present for the diagnosis of HSS while the third phase is the usual ultimate outcome for untreated patients. Comparison with Conventional Angiography. For the clinical diagnosis of BD, the patient must have recurrent oral ulceration with synsrome two of the following clinical manifestations: Only comments seeking to improve the quality and accuracy of information on the Orphanet website are accepted.
Pulmonary artery aneurysms PAA are uncommon, with a diverse differential diagnosis. Multiple pulmonary arterial aneurysms in Behcet’s disease and Hughes-Stovin syndrome.
There is a lack of clear diagnostic criteria and management guidelines for HSS. Multiple pulmonary arterial aneurysms in Behcet’s disease and Hughes-Stovin syndrome. For patients who are not suitable candidates for aggressive surgical intervention, transcatheter arterial embolization, being a less invasive procedure, offers a suitable and effective alternative in HSS [ 8 ].