Hughes–Stovin syndrome: is it incomplete Behçet’s?

Abstract

In this report, we described two male patients with Hughes-Stovin syndrome. The first patient was a 26-year-old male who initially presented with deep vein thrombosis (DVT) in the right lower limb followed shortly by hemoptysis. Pulmonary CT angiography revealed bilateral pulmonary artery aneurysms secondary to underlying pulmonary vasculitis. While the second case was a 16-year-old male patient who initially presented with generalized fits due to sagittal sinus thrombosis and later followed by DVT in the left lower limb. Pulmonary CT angiography showed small pulmonary artery aneurysms with thrombosis of peripheral pulmonary artery branches. Both patients were treated successfully with steroids and immunosuppressive therapy and showed clinical improvement.

Hughes-Stovin Syndrome

“…These signs and symptoms are listed in figure 1[8,16-18]. Patients can have seizures, diplopia and cephalalgia secondary to raised intracranial pressure consequent to cerebral venous sinus thrombosis [1,19]. The raised intracranial pressure also accounts for the papilledema of the optic disc observed in some patients with HSS [20].…”

“…Recurrent phlebitis frequently involves the large vessels resulting in thromboembolism, with even reports of thrombosis of the vena cava, cardiac chambers, jugular vein, iliac vein, femoral vein and dural sinuses [8,10,16,19,24-27]. Patients with thrombosis in the vena cava may have engorged abdominal superficial veins [26].…”

“…Some authors have suggested that HSS may actually be a partially manifested BD owing to their similar findings instead of a novel syndrome [16,19,38,39]. In fact, HSS and BD are the only vasculitides associated with the development of pulmonary artery aneurysms [4,40,41].…”

“…In fact, HSS and BD are the only vasculitides associated with the development of pulmonary artery aneurysms [4,40,41]. HSS has been variably described as "the cardiovascular manifestation of Behcet's disease" [14], "incomplete Behcet's" [19] and "a rare case of Behcet's disease" [42] in literature.…”

Hughes–Stovin Syndrome: A Rare and Often Overlooked Cause of Hemoptysis and Pulmonary Artery Aneurysms

“…The aneurysms can be seen distinctly on pulmonary angiography. 7 The bronchopulmonary fistulas may be obvious on bronchoscopic evaluation. Rarely, aneurysms may form in other lung vasculature.…”