Moderate Hemoptysis Caused by Hughes-Stovin Syndrome

Abstract

Hughes and Stovin first reported a syndrome consisting of multiple pulmonary artery aneurysms and venous thrombosis in 1959. Here, we encountered a 42-year old woman who had hemoptysis revealing a Hughes-Stovin syndrome. Helical computed tomography showed multiple pulmonary artery aneurysms with pulmonary thromboembolism. The patient was treated with steroid therapy, cyclophosphamide and anticoagulation with a good response.

Successful closure of pulmonary artery aneurysm in a patient with Hughes-Stovin syndrome

“…The reported cases ranged between 12 to 48 years of age [5,6] but Korunic et al reported a 70-year-old male patient with HSS [7]. Only seven women have been reported presenting this syndrome thus far [8]. The exact etiology and pathogenesis of HSS remains widely unclear.…”

“…Chest radiography may reveal round opacities and hilar enlargement. Helical computed tomography (HCT) and magnetic resonance imaging have been reported as non-invasive method useful for the diagnosis of PAA [8]. Traditionally, conventional angiography has been regarded as a gold standard for the diagnosis of pulmonary artery aneurysms [2].…”

Severe cardiovascular involvement in Hughes-Stovin syndrome

“…[1][2][3] Typically young men between the second and fourth decades are affected. [4][5][6][7] The prevalence of the disease worldwide is less than 1:1,000,000, [4] making it extremely rare. The clinical symptoms are recurrent fever, chills, peripheral venous thrombosis, multiple pulmonary artery aneurysms, cough, fatigue, dyspnea, chest pain and hemoptysis.…”

“…The clinical symptoms are recurrent fever, chills, peripheral venous thrombosis, multiple pulmonary artery aneurysms, cough, fatigue, dyspnea, chest pain and hemoptysis. [5][6][7] Further, nearly all patients with Behçet's disease present oral mucocutaneous ulcerations in the form of aphthous ulcers. Additionally, patients may present with genital ulcers, erythema nodosum, cutaneous pustular vasculitis or uveitis.…”

Hughes-Stovin syndrome: an unusual cause of pulmonary artery aneurysms

“…Hughes-Stovin syndrome is a rare condition, characterized by the combination of multiple pulmonary artery aneurysms and peripheral venous thrombosis, that mainly affects males (80-90% of cases) between the second and fourth decades of life ( 7 - 11 ) . Although the lesions often affect arteries and veins simultaneously (in 68% of cases), isolated arterial or venous impairments are reported at frequencies of 25% and 7%, respectively ( 9 , 11 ) .…”