Life threatening hemoptysis from Hughes Stovin syndrome: Is it that rare?

Abstract

IntroductionHughes-Stovin syndrome is a life-threatening disorder of unknown etiology. This condition is characterized by vasculitis, deep venous thrombosis and aneurysms that mainly involve the pulmonary arteries resulting in hemoptysis. It has been described in literature less than 40 times. However, we believe it is not very uncommon as it might be diagnosed as pulmonary embolism solely. In such cases, anticoagulation therapy augments the risk of life-threatening hemoptysis.Materials and methodsWe report the case of a 35 years old, Egyptian female patient with Hughes-Stovin syndrome, who initially presented with lower limb deep vein thrombosis and coughing of blood. Anticoagulation regimen for pulmonary embolism was given. This resulted in massive hemoptysis that was successfully controlled by medical therapy.ConclusionAdults who present with venous thrombosis and hemoptoic cough, with no predisposing factors of thrombosis, normal platelet count and coagulation, the possibility of Hughes-Stovin syndrome has to be considered.

Hughes Stovin Syndrome, a Rare Form of Behcet's Disease Presenting as Recurrent Intracardiac Thrombus

“…Intracardiac thrombus appears to be a result of endomyocardial fibrosis, a sequela of vasculitis involving the endocardium, myocardium, or both, and have been histologically described as an organizing thrombus composed of inflammatory cell infiltrates with a mixture of granulocytes and mononuclear inflammatory cells, as seen in our patient [8]. The presence of pulmonary artery aneurysm carries a very poor prognosis and can be associated with significant mortality due to massive hemoptysis secondary to pulmonary artery aneurysm rupture [3,5]. However, early diagnosis and timely intervention can significantly improve the prognosis of patients with HSS and can potentially result in disease remission [3].…”

“…Hughes Stovin syndrome is typically observed in young males between the second and fourth decades of life [1][2][3]. Although pulmonary artery aneurysm can be present in both HSS and Behcet's disease, most reported cases of HSS, including the case presented here, were not found to have the mucocutaneous findings of Behcet's disease [1,5]. The exact etiology and pathogenesis of HSS are unknown, but vasculitis has been proposed as an underlying mechanism [3].…”

Recurrent Haemoptysis Revealing Hughes-Stovin Syndrome

“…Generally, recurrent phlebitis involves the large vessels resulting in thrombus formation. Thrombosis of the vena cava and the right atrium has also been described [5,6] . The aetiology of this syndrome is unknown and its exact pathophysiological mechanism is still uncertain.…”

“…HSS is mostly fatal as a result of massive haemoptysis due to pulmonary/bronchial arterial aneurysm rupture. Likewise, systemic bronchial artery hypertrophy secondary to ischaemia related to the pulmonary artery occlusion could also be the origin of bleeding [5] .…”

Localized Bronchial Hyperemia in Cases of Iatrogenic Hemoptysis: Clinical Presentations and Pathophysiological Mechanisms

“…We suggest that the observed localized vascular changes indicated an active and intense localized vasculitis. In previous reports, bronchoscopic findings were described in 5 cases of HSS, 4 of which had multifocal or diffuse vascular abnormalities described as hypertrophied bronchial varices, easily bleeding edematous bronchial mucosa, or exophytic pulsatile mass and ectatic vessel [19][20][21]. Only one case had localized submucosal lesion compressing and obstructing lobar bronchus [20].…”