Hughes-Stovin syndrome: A rare cause of hemoptysis

Abstract

Multiple pulmonary artery aneurysms are seen along with venous thrombosis in Hughes-Stovin syndrome, which many investigators believe is an incomplete form of Behcet's disease. We present a case of hemoptysis with multiple pulmonary artery aneurysms, femoral vein thrombosis, and oral ulcers with emphasis on its CT features.

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

“…The distribution of the vascular component of the syndrome is as follows: arterial (7%), venous (25%) or both (68%) [14]. Some authors have proposed that HSS may actually be a partially manifested Behçet's disease (BD) based on their similar findings [12,15,16]. In fact, HSS and BD are the only vasculitides associated with the development of pulmonary artery aneurysms yet most HSS patients do not have typical manifestations of BD such as oral and genital ulcers, uveitis or arthralgia.…”

“…Today, in fact, multi-detector row helical CT angiography offers more precise visualization of large systemic arteries than does conventional angiography [2], and is considered the diagnostic method of choice in most of the patients. Classic histopathologic findings of HSS [1,2,12] include diffuse dilatation and partial occlusion of the aneurysmal arteries, perivascular infiltration that is predominantly lymphomonocytic and diffuse proliferative sclerosis. In the pulmonary veins, fibrosis and muscular medial thickening is seen.…”

“…Actually, weakening of the arterial wall from the subsequent inflammation is the likely mechanism [11]. The clinical course of HSS can be categorized into three different phases [3,4]: a) symptoms of thrombophlebitis, b) formation of large pulmonary and/or bronchial aneurysms and c) aneurysmal rupture leading to massive hemoptysis Patients can have seizures, diplopia and cephalalgia secondary to raised intracranial pressure consequent to cerebral venous sinus thrombosis [1,12] akin to the patient reported by Chalazonitis et al [13]. About 25% of patients with HSS develop thromboembolism, arterial aneurysms and vascular occlusions.…”

Hughes-Stovin Syndrome and Massive Hemoptysis: A Management Challenge

“…Hemoptysis can either arise later or be observed at the initial presentation. 4 The aneurysms usually involve the pulmonary and/or the bronchial arteries, although the systemic circulation can be affected too. 5 They can present with vascular thromboembolism, arterial aneurysms, arterial and venous occlusions, non-specific vasculitis and even thrombophlebitis.…”

Behçet's disease with major vascular involvement

“…Imaging of the pulmonary arteries should be obtained to screen for aneurysms in these cases. Although the constellation of DVT with pulmonary artery aneurysms has been described as Hughes-Stovin syndrome, significant doubt has emerged as to its existence as an independent entity rather than undiagnosed BD 18 19. A ‘Hughes-Stovin’ presentation in a young Middle or Far Eastern (mostly male) patient with a history of recurrent oral ulcers is highly suggestive—and virtually diagnostic—of BD, even at a point when other diagnostic criteria for BD are either not present or cannot be elicited from the patient.…”