Letters to the Editor
Hughes–Stovin Syndrome: A Rare and Often Overlooked Cause of Hemoptysis and Pulmonary Artery Aneurysms Shailendra Kapoor1 1 Mechanicsville, Virginia, United States
Thorac Cardiovasc Surg 2014;62:456–457.
Address for correspondence Shailendra Kapoor, 7487 Sherwood Crossing Place # 302, Mechanicsville, VA 23111, United States (e-mail: [email protected]).
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 hyperhomocysteinemia.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.
received November 30, 2013 accepted December 4, 2013 published online March 3, 2014
© 2014 Georg Thieme Verlag KG Stuttgart · New York
References 1 Papaioannou V, Mikroulis D, Chrysafis I, Fotakis S, Pneumatikos I.
2
3
4
5
6
7
Hemoptysis due to a mycotic pulmonary artery aneurysm in an injecting drug user. Thorac Cardiovasc Surg 2014;62:453–455 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 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 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 Chalazonitis AN, Lachanis SB, Mitseas P, et al. Hughes-Stovin syndrome: a case report and review of the literature. Cases J 2009;2:98 Kim HO, Kim HC, Park Y, et al. A case of Hughes-Stovin syndrome associated with hyperhomocysteinemia. Clin Rheumatol 2010; 29(7):807–809 Emad Y, Ragab Y, Shawki Ael-H, Gheita T, El-Marakbi A, Salama MH. Hughes-Stovin syndrome: is it incomplete Behçet’s? Report of
DOI http://dx.doi.org/ 10.1055/s-0034-1364258. ISSN 0171-6425.
This document was downloaded for personal use only. Unauthorized distribution is strictly prohibited.
456
Letters to the Editor 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 throm-
457
bosis (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
Vasilios Papaioannou1 1 Department of Intensive Care Unit, Democritus University of Thrace,
Dragana, Alexandroupolis, Greece
We appreciate the interest of the author in our recently published case report on “hemoptysis due to a mycotic pulmonary artery aneurysm in an injecting drug user.” The author, after a presentation of the major characteristics of the Hughes–Stovin syndrome (HSS), suggests that our case could also be associated with this particular manifestation of Behçet disease (BD). BD is by definition a chronic, relapsing autoinflammatory condition characterized by recurrent episodes of systemic inflammation. Vascular involvement may include both veins and arteries with any size, whereas the abdominal aorta has been reported as the most common site of aneurysm development. On the contrary, pulmonary artery aneurysms (PAAs) have an associated rate of 1 to 10%.1 According to the original theory of Hughes and Stovin,2 degenerative bronchial arteries lead to changes in vasa vasorum of pulmonary arteries with subsequent multiple aneurysm development. In this respect, contrast-enhanced magnetic resonance angiography (MRA) and three-dimensional volume rendering analysis can visualize the presence of distorted or dilated bronchial arteries even before aneurysm formation.3 In our case, there were no multiple PAAs on contrastenhanced computed tomography (CT) images and the patient was suffering from a severe pulmonary infection associated with groin abscess, due to Staphylococcus aureus. The patient also developed severe sepsis with both respiratory and coagulation systems involvement. Although infection may trigger an
Address for correspondence Vasilios Papaioannou, MD, MSc, PhD, Department of Intensive Care Unit, Democritus University of Thrace, Dragana 68100, Alexandroupolis, Greece (e-mail: [email protected]).
inflammatory vascular response in patients with BD, in autoimmune disorders there is, by definition, a lack of known specific antigens and antibodies. Thus, the hypothesis of PAA formation through septic microemboli with local necrotic inflammation seems much more likely. In addition, bronchoscopy was normal excluding the presence of bronchopulmonary fistulas that might be present in cases with HSS, whereas CT scan did not reveal dysplastic bronchial arteries. In conclusion, we also suggest that in any case with recurrent episodes of phlebitis and thrombus formation without known causes, as well as dysplastic bronchial arteries with pulmonary aneurysms found in MRA or CT pulmonary angiograms, both the radiologist and the caring clinician should raise suspicion of pulmonary vasculitis possibly associated with HSS.
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
Thoracic and Cardiovascular Surgeon
Vol. 62
No. 5/2014
This document was downloaded for personal use only. Unauthorized distribution is strictly prohibited.
Reply by the Authors of the Original Article
Copyright of Thoracic & Cardiovascular Surgeon is the property of Georg Thieme Verlag Stuttgart and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use.
Hughes–Stovin Syndrome: A Rare and Often Overlooked Cause of Hemoptysis and Pulmonary Artery Aneurysms Shailendra Kapoor1 1 Mechanicsville, Virginia, United States
Thorac Cardiovasc Surg 2014;62:456–457.
Address for correspondence Shailendra Kapoor, 7487 Sherwood Crossing Place # 302, Mechanicsville, VA 23111, United States (e-mail: [email protected]).
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 hyperhomocysteinemia.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.
received November 30, 2013 accepted December 4, 2013 published online March 3, 2014
© 2014 Georg Thieme Verlag KG Stuttgart · New York
References 1 Papaioannou V, Mikroulis D, Chrysafis I, Fotakis S, Pneumatikos I.
2
3
4
5
6
7
Hemoptysis due to a mycotic pulmonary artery aneurysm in an injecting drug user. Thorac Cardiovasc Surg 2014;62:453–455 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 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 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 Chalazonitis AN, Lachanis SB, Mitseas P, et al. Hughes-Stovin syndrome: a case report and review of the literature. Cases J 2009;2:98 Kim HO, Kim HC, Park Y, et al. A case of Hughes-Stovin syndrome associated with hyperhomocysteinemia. Clin Rheumatol 2010; 29(7):807–809 Emad Y, Ragab Y, Shawki Ael-H, Gheita T, El-Marakbi A, Salama MH. Hughes-Stovin syndrome: is it incomplete Behçet’s? Report of
DOI http://dx.doi.org/ 10.1055/s-0034-1364258. ISSN 0171-6425.
This document was downloaded for personal use only. Unauthorized distribution is strictly prohibited.
456
Letters to the Editor 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 throm-
457
bosis (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
Vasilios Papaioannou1 1 Department of Intensive Care Unit, Democritus University of Thrace,
Dragana, Alexandroupolis, Greece
We appreciate the interest of the author in our recently published case report on “hemoptysis due to a mycotic pulmonary artery aneurysm in an injecting drug user.” The author, after a presentation of the major characteristics of the Hughes–Stovin syndrome (HSS), suggests that our case could also be associated with this particular manifestation of Behçet disease (BD). BD is by definition a chronic, relapsing autoinflammatory condition characterized by recurrent episodes of systemic inflammation. Vascular involvement may include both veins and arteries with any size, whereas the abdominal aorta has been reported as the most common site of aneurysm development. On the contrary, pulmonary artery aneurysms (PAAs) have an associated rate of 1 to 10%.1 According to the original theory of Hughes and Stovin,2 degenerative bronchial arteries lead to changes in vasa vasorum of pulmonary arteries with subsequent multiple aneurysm development. In this respect, contrast-enhanced magnetic resonance angiography (MRA) and three-dimensional volume rendering analysis can visualize the presence of distorted or dilated bronchial arteries even before aneurysm formation.3 In our case, there were no multiple PAAs on contrastenhanced computed tomography (CT) images and the patient was suffering from a severe pulmonary infection associated with groin abscess, due to Staphylococcus aureus. The patient also developed severe sepsis with both respiratory and coagulation systems involvement. Although infection may trigger an
Address for correspondence Vasilios Papaioannou, MD, MSc, PhD, Department of Intensive Care Unit, Democritus University of Thrace, Dragana 68100, Alexandroupolis, Greece (e-mail: [email protected]).
inflammatory vascular response in patients with BD, in autoimmune disorders there is, by definition, a lack of known specific antigens and antibodies. Thus, the hypothesis of PAA formation through septic microemboli with local necrotic inflammation seems much more likely. In addition, bronchoscopy was normal excluding the presence of bronchopulmonary fistulas that might be present in cases with HSS, whereas CT scan did not reveal dysplastic bronchial arteries. In conclusion, we also suggest that in any case with recurrent episodes of phlebitis and thrombus formation without known causes, as well as dysplastic bronchial arteries with pulmonary aneurysms found in MRA or CT pulmonary angiograms, both the radiologist and the caring clinician should raise suspicion of pulmonary vasculitis possibly associated with HSS.
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
Thoracic and Cardiovascular Surgeon
Vol. 62
No. 5/2014
This document was downloaded for personal use only. Unauthorized distribution is strictly prohibited.
Reply by the Authors of the Original Article
Copyright of Thoracic & Cardiovascular Surgeon is the property of Georg Thieme Verlag Stuttgart and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use.
