Hughes–Stovin Syndrome: A Rare and Often Overlooked Cause of Hemoptysis and Pulmonary Artery Aneurysms
30 November 2013
04 December 2013
03 March 2014 (online)
I read with great interest the recent article by Papaioannou et al. Interestingly, one rare cause of pulmonary artery aneurysms and hemoptysis that is often overlooked is Hughes–Stovin syndrome (HSS).
The general consensus is that HSS is an incomplete type of Behçet disease. Multiple pulmonary artery aneurysms coexisting with peripheral vein thrombosis are the characteristic feature of the syndrome. HSS has been diagnosed in individuals as young as 11 years of age. It has also been reported in individuals as old as 48 years of age. A greater preponderance of the disease is seen in males. Interestingly, de Vries et al recently reported the case of a male child with HSS who presented as a variant of “polyarteritis nodosa.”
Patients with HSS usually present with a history of dyspnea and recurrent hemoptysis. Cough and chest pain are also typically present. Recurrent oral ulcerations are usually present. Genital ulcers may be present simultaneously. Low-grade fever is usually present. Patients usually have a coexisting peripheral vein thrombosis such as a dural venous sinus thrombosis or an inferior vena caval thrombosis. Femoral vein thrombi are seen the most often. Interestingly, HSS may be associated with a myriad of other rare conditions. For instance, Kim et al recently reported the case of a patient with HSS with concurrent hyper-homocysteinemia. Thrombus formation in the pulmonary artery aneurysms may further complicate the clinical scenario of HSS.
Computed tomographic imaging reveals multiple pulmonary artery aneurysms. The aneurysms can be seen distinctly on pulmonary angiography. The bronchopulmonary fistulas may be obvious on bronchoscopic evaluation. Rarely, aneurysms may form in other lung vasculature. For instance, Herb et al recently reported a 25-year-old man with HSS and concurrent aneurysms of the bronchial arteries.
Initial management usually involves the use of corticosteroids. Cyclophosphamide therapy is indicated in steroid recalcitrant cases. Alí-Munive et al have also recently reported the successful use of azathioprine therapy. Interestingly, colchicine has also been used successfully for the management of HSS. The hemoptysis may be so severe in some patients that ventilatory support may be necessary. Tzilalis et al have also recently reported the successful management of HSS by using Amplatzer vascular plug embolization. In general, anticoagulants should be avoided in patients with HSS. Massive hemoptysis may require lobectomy in some patients.
An overall poor prognosis is seen in patients with HSS. HSS should be kept high on the differential in young males presenting with hemoptysis in the setting of a peripheral venous thrombus.
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