Hughes-Stovin syndrome is a rare syndrome characterized by peripheral venous thrombosis, cerebral venous sinus thrombosis and recurrent pulmonary artery aneurysms (PAA). Aneurysms in the systemic circulation can also be seen. We report the first case of Hughes-Stovin syndrome with coronary artery aneurysm.
A 25 year old male presented to an outside hospital with a history of hemoptysis, fatigue and dyspnea for 4 months. Chest CT scan revealed a large right upper lobe PAA. Due to uncontrollable hemoptysis, patient had a right upper lobe lobectomy. Post-operatively he developed ventilator dependent respiratory failure and deep venous thrombosis (DVT) in all four extremities. Hemoptysis recurred, and repeat CT scan showed new PAA in right lower lobe and left lung. Patient was transferred to our institution. On arrival, he complained of intermittent hemoptysis, dyspnea and weakness. He denied joint pain, oral or genital ulcers, rash, or visual changes. He had a history of congenital hydrocephalus treated with a ventriculo-peritoneal shunt and dural sinus thrombosis requiring warfarin anticoagulation. He was a non-smoker and did not use illicit drugs. Physical exam and basic laboratory studies were normal.Rheumatologic workup was significant for weakly positive anti-histone antibodies and elevated C-reactive protein. Chest CT scan showed four PAA, the largest of which was partially thrombosed. There was a 2.2 cm vascular lesion in the posterior aspect of the left ventricle consistent with a coronary artery aneurysm. Based on combination of PAA, DVT, and sagittal sinus thrombosis Hughes-Stovin syndrome was diagnosed. He was started on intravenous methylprednisone 250mg Q6H for three days and received cyclophosphamide 750 mg loading dose. His hemoptysis decreased significantly within 72 hours He was discharged on oral cyclophosphamide and prednisone. At two weeks follow up he was asymptomatic.
Hughes-Stovin syndrome is a rare disorder of unknown etiology first described in 1959. The authors described four males with DVT, cerebral venous sinus thrombosis and PAA. They hypothesized the PAA were due to degenerative changes in bronchial arteries or infected emboli from low virulence organisms. Hughes-Stovin syndrome has been postulated to be a variant of Behcet’s disease due to some similarities in clinical and histopathological findings. Systemic vasculitis has been suggested as etiology due to involvement of systemic circulation. It commonly occurs in young men. Clinical features include recurrent fever, chills, cough and hemoptysis that can be massive and fatal. Other features include both superficial and deep venous thrombosis, signs of elevated intracranial pressure and pulmonary hypertension. Aneurysms in systemic circulation have been reported in multiple case reports. Our patient is the first reported case of coronary aneurysm in Hughes-Stovin syndrome.Hughes-Stovin syndrome should be suspected in any young patient with hemoptysis and PAA in the absence of clinical features of Behcet’s disease. Chest CT scan is obtained to detect PAA.Given its similarities with Behcet’s diseases; treatment with corticosteroids and immunosuppressive agents has been suggested.
1) Hughes-Stovin syndrome is a rare disorder characterized by PAA and venous thrombosis. 2) Systemic vasculitis has been suggested as etiology, and thus it can affect any systemic circulation. In our patient it caused a coronary artery aneurysm. 3) The combination of corticosteroids and Cyclophosphamide appears to be effective treatment for this rare and potentially fatal disorder.
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