Article, Pulmonology

Hemoptysis caused by Hughes-Stovin syndrome

Correspondence

Hemoptysis caused by Hughes-Stovin syndrome

To the Editor,

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 treat- ments 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 hemop- tysis 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. 1). A subsequent computed tomography (CT) of chest showed pulmonary artery aneurysms in the right lower and left lower lobes with mural thrombi (Fig. 2). Inferior vena cava thromboses were also found in this CT study. A pulmonary Ventilation/perfusion scan was per- formed, 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 concom- itant 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. 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 [1]. Since that time, several cases of the Hughes-Stovin syndrome have been published [2-5]. 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) [6,7]. 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) [8,9]. 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 syn- drome have unstable pulmonary artery aneurysms, which may result in massive hemoptysis. However, some patients have concomitant pulmonary embolisms within the aneur- ysms, probably because of an inflammatory response of the vascular Endothelial cells [8-10].

Imaging studies for diagnosis of pulmonary aneurysm are straightforward, such as a chest radiograph or a CT [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 [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 [5,6,13]. 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 con-

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Fig. 1 Chest radiography reveals rounded opacities in bilateral lung fields.

tinued to have hemoptysis [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 neces- sitate mechanical ventilatory support [5]. A consultation with cardiothoracic surgeon should be obtained immediate- ly. The affected segments of the lung should be resected if applicable, predicated on the preoperative MRA or angio- graphic findings [12]. If the aneurysms are identified in the bronchial arteries, bleeding can be treated by embolization of bronchial arteries [4,14].

Fig. 2 CT of the chest shows pulmonary artery aneurysms in the right lower and left lower lobes with mural thrombus formation.

Correspondence

Fig. 3 MRA (enhanced axial and coronal T1-weighted image) discloses 5 aneurysms of pulmonary arteries at the right upper, right middle, right lower, left lingular, and left lower lobes, respectively.

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 immu- nosuppressant and aggressive surgical intervention are imperative for the life-threatening hemoptysis.

References

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Chu-Lin Tsai MD Tsung-Chien Lu MD Kuang-Chau Tsai MD Wen-Jone Chen, MD PhD

Department of Emergency Medicine National Taiwan University Hospital

Taipei 100, Taiwan E-mail address: [email protected]