Hemoptysis is a common complaint that emergency physicians encounter. Most cases are minor or self-limited; however, massive hemoptysis occasionally occurs and can be life-threatening. Patients with Hughes-Stovin syndrome can present with massive hemoptysis from pulmonary artery aneurysms and pulmonary embolisms, and specific treatments are required for this potentially lethal condition. Here we report a unique case and discuss the clinical implications for emergency physicians.

A 34-year-old man, with previously healthy status, presented to the emergency department (ED) with hemoptysis for 1 week. The amount of hemoptysis increased gradually in 1 week's duration despite antitussive treatment. He denied taking any drug that might predispose to bleeding. Physical examinations were unremarkable except superficial venous engorgement over his abdomen and legs. No ulcer was found in his oral cavity or genital region. His platelet count and coagulation tests were within normal limits. A chest radiography revealed rounded opacities in bilateral lung fields (Fig. 1Fig. 1). A subsequent computed tomography (CT) of chest showed pulmonary artery aneurysms in the right lower and left lower lobes with mural thrombi (Fig. 2Fig. 2). Inferior vena cava thromboses were also found in this CT study. A pulmonary ventilation/perfusion scan was performed, which showed bilateral and well-defined ventilation/perfusion mismatched areas, suggestive of a high probability of pulmonary embolism. However, anticoagulants were not given for the pulmonary embolism because of his concomitant hemoptysis condition. Finally, a rare diagnosis of systemic vasculitis, Hughes-Stovin syndrome, was made on the basis of multiple pulmonary artery aneurysms and inferior vena cava thromboses in such a young patient. A magnetic resonance angiography (MRA) was performed to better understand the morphology of pulmonary artery aneurysms, and it disclosed 5 aneurysms of the pulmonary arteries at the right upper, right middle, right lower, left lingular, and left lower lobes, respectively (Fig. 3Fig. 3). A consultation with a cardiothoracic surgeon was obtained and lung transplantation was suggested for the multifocal aneurysms. The hemoptysis resolved with intravenous methylprednisolone treatment. The patient has been well, without a recurrence of hemoptysis, for 1 year.

Hughes and Stovin first described a syndrome consisting of multiple pulmonary aneurysms and peripheral venous thrombosis in 1959 [1x[1]Hughes, J.P. and Stovin, P.G.I. Segmental pulmonary artery aneurysms. Thorax. 1961; 16: 364–371

CrossRef | PubMedSee all References][1]. Since that time, several cases of the Hughes-Stovin syndrome have been published [[2]x[2]Teplick, J.G., Haskin, M.E., and Nedwich, A. The Hughes-Stovin syndrome. Case report. Radiology. 1974; 113: 607–608

PubMedSee all References, [3]x[3]Roberts, D.H., Jimenez, J.F., and Golladay, E.S. Multiple pulmonary artery aneurysms and peripheral venous thromboses—the Hughes Stovin syndrome. Report of a case in a 12-year-old boy and a review of the literature. Pediatr. Radiol. 1982; 12: 214–216

CrossRef | PubMed | Scopus (9)See all References, [4]x[4]Herb, S., Hetzel, M., Hetzel, J. et al. An unusual case of Hughes-Stovin syndrome. Eur. Respir. J. 1998; 11: 1191–1193

CrossRef | PubMed | Scopus (19)See all References, [5]x[5]Riantawan, P., Yodtasurodom, C., Chotivatanapong, T. et al. Hughes-Stovin syndrome: a case report and review of the literature. J. Med. Assoc. Thai. 1999; 82: 312–316

PubMedSee all References]. Hughes-Stovin syndrome might be, in fact, a cardiovascular manifestation of Behcet disease or the incomplete type of Behcet disease (isolated pulmonary presentation of Behcet disease) [[6]x[6]Durieux, P., Bletry, O., Huchon, G. et al. Multiple pulmonary arterial aneurysms in Behcet's disease and Hughes-Stovin syndrome. Am. J. Med. 1981; 71: 736–741

Abstract | Full Text PDF | PubMed | Scopus (85)See all References, [7]x[7]Francois, M.F. Is Hughes-Stovin syndrome a particular expression of Behcet's disease?. Chest. 1983; 83: 288

CrossRef | PubMedSee all References]. Arterial involvement in Behcet disease was expressed by aneurysms that can involve all arterial territories with a preference for the pulmonary arteries (Hughes-Stovin syndrome) [[8]x[8]Higuchi, M., Kitamura, S., and Terada, I. An autopsy case of multiple intrapulmonary artery aneurysms with systemic thromboendophlebitis, the “Hughes-Stovin syndrome”. Acta Pathol. Jpn. 1969; 19: 69–79

PubMedSee all References, [9]x[9]Kinjo, M., Tanaka, K., Ishimaru, S. et al. Hughes-Stovin syndrome. Report of a female autopsy case and review of the literature. Acta Pathol. Jpn. 1978; 28: 335–344

PubMedSee all References]. As for the venous involvement, recurrent phlebitis commonly involves the large vessels and results in thrombus formation. Like our case, he presented with engorged abdominal superficial veins, suggestive of vena cava thrombosis. Most patients with Hughes-Stovin syndrome have unstable pulmonary artery aneurysms, which may result in massive hemoptysis. However, some patients have concomitant pulmonary embolisms within the aneurysms, probably because of an inflammatory response of the vascular endothelial cells [[8]x[8]Higuchi, M., Kitamura, S., and Terada, I. An autopsy case of multiple intrapulmonary artery aneurysms with systemic thromboendophlebitis, the “Hughes-Stovin syndrome”. Acta Pathol. Jpn. 1969; 19: 69–79

PubMedSee all References, [9]x[9]Kinjo, M., Tanaka, K., Ishimaru, S. et al. Hughes-Stovin syndrome. Report of a female autopsy case and review of the literature. Acta Pathol. Jpn. 1978; 28: 335–344

PubMedSee all References, [10]x[10]Bowman, S. and Honey, M. Pulmonary arterial occlusions and aneurysms: a forme fruste of Behcet's or Hughes-Stovin syndrome. Br. Heart J. 1990; 63: 66–68

CrossRef | PubMedSee all References].

Imaging studies for diagnosis of pulmonary aneurysm are straightforward, such as a chest radiograph or a CT [11x[11]Ammann, M.E., Karnel, F., Olbert, F. et al. Radiologic findings in the diagnosis of Hughes-Stovin syndrome. Am. J. Roentgenol. 1991; 157: 1353–1354

CrossRef | PubMedSee all References][11]. For better evaluation of the aneurysms in Hughes-Stovin syndrome, conventional pulmonary angiography remains the best tool. However, it cannot be performed in patients with thromboses in the inferior vena cava, because the thromboses impede catheter passage. In these cases, contrast-enhanced 3-dimensional MRA may be a substitute for visualizing the aneurysms, as in our case [12x[12]Balci, N.C., Semelka, R.C., Noone, T.C. et al. Multiple pulmonary aneurysms secondary to Hughes-Stovin syndrome: demonstration by MR angiography. J. Magn. Reson. Imaging. 1998; 8: 1323–1325

CrossRef | PubMedSee all References][12].

Once the Hughes-Stovin syndrome is recognized in the ED, a prompt treatment with immunosuppressant, either systemic corticosteroids or cytotoxic agents, confers clinical improvement and stabilizing of the pulmonary artery aneurysms in most patients [[5]x[5]Riantawan, P., Yodtasurodom, C., Chotivatanapong, T. et al. Hughes-Stovin syndrome: a case report and review of the literature. J. Med. Assoc. Thai. 1999; 82: 312–316

PubMedSee all References, [6]x[6]Durieux, P., Bletry, O., Huchon, G. et al. Multiple pulmonary arterial aneurysms in Behcet's disease and Hughes-Stovin syndrome. Am. J. Med. 1981; 71: 736–741

Abstract | Full Text PDF | PubMed | Scopus (85)See all References, [13]x[13]Efthimiou, J., Johnston, C., Spiro, S.G. et al. Pulmonary disease in Behcet's syndrome. QJM. 1986; 58: 259–280

PubMedSee all References]. However, serious hemoptysis occasionally recurs despite these treatments. Moreover, if the pulmonary embolism is present in the aneurysms, this condition may pose a therapeutic dilemma in using anticoagulants. Anticoagulation prevents the progression of pulmonary embolism, yet it increases the risk of hemoptysis. A study of isolated pulmonary Behcet disease presenting with hemoptysis showed that 4 patients treated with anticoagulants for pulmonary embolism continued to have hemoptysis [13x[13]Efthimiou, J., Johnston, C., Spiro, S.G. et al. Pulmonary disease in Behcet's syndrome. QJM. 1986; 58: 259–280

PubMedSee all References][13]. One of them subsequently died after massive hemoptysis, despite good anticoagulant control. Based on these observations, we think that using anticoagulants should be reserved only for patients with embolisms in the main pulmonary artery that causes hemodynamic instability.

If the pulmonary artery aneurysms rupture in the ED and life-threatening massive hemoptysis follows, it may necessitate mechanical ventilatory support [5x[5]Riantawan, P., Yodtasurodom, C., Chotivatanapong, T. et al. Hughes-Stovin syndrome: a case report and review of the literature. J. Med. Assoc. Thai. 1999; 82: 312–316

PubMedSee all References][5]. A consultation with cardiothoracic surgeon should be obtained immediately. The affected segments of the lung should be resected if applicable, predicated on the preoperative MRA or angiographic findings [12x[12]Balci, N.C., Semelka, R.C., Noone, T.C. et al. Multiple pulmonary aneurysms secondary to Hughes-Stovin syndrome: demonstration by MR angiography. J. Magn. Reson. Imaging. 1998; 8: 1323–1325

CrossRef | PubMedSee all References][12]. If the aneurysms are identified in the bronchial arteries, bleeding can be treated by embolization of bronchial arteries [[4]x[4]Herb, S., Hetzel, M., Hetzel, J. et al. An unusual case of Hughes-Stovin syndrome. Eur. Respir. J. 1998; 11: 1191–1193

CrossRef | PubMed | Scopus (19)See all References, [14]x[14]Mahlo, H.R., Elsner, K., Rieber, A. et al. New approach in the diagnosis of and therapy for Hughes-Stovin syndrome. Am. J. Roentgenol. 1996; 167: 817–818

CrossRef | PubMedSee all References].

In summary, patients with Hughes-Stovin syndrome can present to the ED with unstable pulmonary aneurysms and hemoptysis. Although massive hemoptysis is infrequent, it carries a very serious prognosis. This report underscores the potentially lethal condition of Hughes-Stovin syndrome for emergency physicians. Appropriate treatments with immunosuppressant and aggressive surgical intervention are imperative for the life-threatening hemoptysis.