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

I read with great interest the recent article by Papaioannou et al.[1] 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.[1] HSS has been diagnosed in individuals as young as 11 years of age.[2] It has also been reported in individuals as old as 48 years of age. A greater preponderance of the disease is seen in males.[3] Interestingly, de Vries et al recently reported the case of a male child with HSS who presented as a variant of “polyarteritis nodosa.”[4]

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.[5] 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.[6] 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.[7] 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.[8]

Initial management usually involves the use of corticosteroids. Cyclophosphamide therapy is indicated in steroid recalcitrant cases.[9] Alí-Munive et al have also recently reported the successful use of azathioprine therapy.[10] 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.[11] 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.

• References

• 1 Papaioannou V, Mikroulis D, Chrysafis I, Fotakis S, Pneumatikos I. Hemoptysis due to a mycotic pulmonary artery aneurysm in an injecting drug user. Thorac Cardiovasc Surg 2014; 62: 453-455
• 2 Al-Jahdali H. Massive hemoptysis and deep venous thrombosis presenting in a woman with Hughes-Stovin syndrome: a case report. J Med Case Reports 2010; 4: 109
• 3 Choh NA, Jehangir M, Mir KM, Kuchay S, Wani NA. Hughes-Stovin syndrome: a rare cause of hemoptysis. Lung India 2011; 28 (4) 285-286
• 4 de Vries W, Koppelman GH, Roofthooft MT, Bootsma H, Leijsma MK, Armbrust W. Pulmonary medium vessel vasculitis in an 11 year old boy: Hughes Stovin syndrome as a variant of polyarteritis nodosa?. Pediatric Rheumatology 2011; 9: 1-4
• 5 Chalazonitis AN, Lachanis SB, Mitseas P , et al. Hughes-Stovin syndrome: a case report and review of the literature. Cases J 2009; 2: 98
• 6 Kim HO, Kim HC, Park Y , et al. A case of Hughes-Stovin syndrome associated with hyperhomocysteinemia. Clin Rheumatol 2010; 29 (7) 807-809
• 7 Emad Y, Ragab Y, Shawki Ael-H, Gheita T, El-Marakbi A, Salama MH. Hughes-Stovin syndrome: is it incomplete Behçet's? Report of two cases and review of the literature. Clin Rheumatol 2007; 26 (11) 1993-1996
• 8 Herb S, Hetzel M, Hetzel J, Friedrich J, Weber J. An unusual case of Hughes-Stovin syndrome. Eur Respir J 1998; 11 (5) 1191-1193
• 9 Lee J, Noh JW, Hwang JW , et al. Successful cyclophosphamide therapy with complete resolution of pulmonary artery aneurysm in Hughes-Stovin syndrome patient. Clin Rheumatol 2008; 27 (11) 1455-1458
• 10 Alí-Munive A, Varón H, Maldonado D, Torres-Duque C. Giant aneurysms of the pulmonary artery and peripheral venous thrombosis (Hughes-Stovin syndrome): regression with immunosuppressant therapy [in Spanish]. Arch Bronconeumol 2001; 37 (11) 508-510
• 11 Tzilalis VD, Vourliotakis G, Tsironis IA, Tsiligiris VD, Brountzos EN. Use of an Amplatzer vascular plug in embolization of a pulmonary artery aneurysm in a case of Hughes-Stovin syndrome: a case report. J Med Case Reports 2011; 5: 1-3

• References

• 1 Uzun O, Akpolat T, Erkan L. Pulmonary vasculitis in Behcet disease: a cumulative analysis. Chest 2005; 127 (6) 2243-2253
• 2 Hughes JP, Stovin PGI. Segmental pulmonary artery aneurysms with peripheral venous thrombosis. Br J Dis Chest 1959; 53 (1) 19-27
• 3 Ketchum ES, Zamanian RT, Fleischmann D. CT angiography of pulmonary artery aneurysms in Hughes-Stovin syndrome. AJR Am J Roentgenol 2005; 185 (2) 330-332