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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Learning points for clinicians Pulmonary artery aneurysms PAA have a diverse differential diagnosis. In solitary peripheral PAA, we recommend operation or embolization for rapidly growing or high-pressure aneurysms.
Multiple, small PAA warrant early immunosuppression. Anticoagulation is typically contraindicated by bleeding risk. Pulmonary artery aneurysms PAA are uncommon, with a diverse differential diagnosis. Although most are asymptomatic at diagnosis, fatal aneurysm rupture occurs in one-third and dissection in one-fifth. A year-old male from Missouri, USA was admitted with haemoptysis following 1 month of intermittent fevers, non-productive cough and weight loss.
Past medical history was significant for recurrent huyhes ulcers. Syndromd examination found fever, tachycardia and reduced air entry to his right lung base.
Ophthalmological examination excluded uveitis, vitritis or retinal vasculitis and he did not have genital ulceration or skin manifestations of BD. He developed superficial thrombophlebitis at venous access sites but no pathergy.
There was no huhhes trauma or features of connective tissue disorders. Chest x-ray demonstrated a right lower zone circumscribed lesion. He was treated with antibiotics for community-acquired pneumonia.
CT angiogram found normal mesenteric, hepatic and renal vasculature. Echocardiogram excluded pulmonary artery hypertension PAH and structural cardiac disease. Extensive microbiological investigation found no evidence of infection. Immunological testing found a weak-positive ANA 1: PAA resection by thoracotomy and lower lobe basal segmentectomy was performed.
He returned to America post-operatively for immunosuppressive treatment. Tissue pathology demonstrated necrotising lymphocytic vasculitis with pulmonary infarction. Based on clinical and histopathological findings, without meeting BD criteria, he was diagnosed with HSS.
Both conditions predominantly affect syndrom males, present with fever and haemoptysis, and involve the right lower lobar artery. Immunosuppression may stabilize or promote regression of PAA. However, long-term outcome data does not exist and the procedures risk aneurysm rupture, distal infarction, and procedural failure, particularly for larger aneurysms. atovin
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 sjndrome embolization procedures. Oxford University Press is a department of the University of Oxford. It furthers the University’s objective of excellence in research, scholarship, and education by publishing worldwide.
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Hughes–Stovin syndrome – Wikipedia
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