J Thorac Cardiovasc Surg 2004;127:590-592
© 2004 The American Association for Thoracic Surgery
Pulmonary artery pseudoaneurysm in a patient with Behçet disease
Yong Han Yoon, MDa,*,
Kwang Ho Kim, MDa,
Wan Ki Baek, MDa,
Jung Taek Kim, MDa,
Kuk Hee Shon, MDa,
Young Sam Kim, MDa,
Hae Seung Han, MDb,
Jae Hwa Cho, MDc
a Department of Cardiovascular Thoracic Surgery, Inha University Hospital, College of Medicine, Inha University, Incheon, South Korea
b Department of Pathology, Inha University Hospital, College of Medicine, Inha University, Incheon, South Korea
c Department of Internal Medicine, Inha University Hospital, College of Medicine, Inha University, Incheon, South Korea
Received for publication August 29, 2003; accepted for publication September 30, 2003.
* Address for reprints: Yong Han Yoon, MD, Department of Thoracic and Cardiovascular Surgery, Inha University Hospital, 7-206, 3-ga, Shinheung-dong, Jung-gu, Incheon 400-103, South Korea
yoonkwon{at}inha.ac.kr
Behçet disease is a rare and chronic disorder of unknown cause characterized by the inflammation of blood vessels; pulmonary involvement occurs in only about 1% to 7% of patients.1-4 Of the pulmonary manifestations, the pulmonary arteries are second to the aorta as the most common site of arterial involvement. Pulmonary artery aneurysm, in particular, has a poor prognosis and is one of the leading causes of death in patients with Behçet disease. Mean survival after the onset of hemoptysis was reported to be about 10 months in one study of patients with Behçet disease and pulmonary artery aneurysm.5 We report a case involving a young female patient who presented with a variety of symptoms, leading to a diagnosis of Behçet disease and treatment for pulmonary artery pseudoaneurysm after appropriate investigations and surgical intervention. After a right lower lobe lobectomy, no further hemoptysis and skin lesions have occurred 20 months postoperatively.
Clinical summary
A 35-year-old woman was admitted to Inha University Hospital, Incheon, Korea, because of high fever and cough for the previous 2 months. For the previous 3 years, she had recurrent episodes of oral ulceration. A chest radiograph taken 2 months before admission showed a mass-like lesion in the right lower lung field. Physical examination revealed several small, ulcerated lesions on her external genitalia. However, her breath sounds were clean, and heart sounds were regular and without murmur. Pathergy test results were positive, but the results of other blood tests were within normal limits. The chest radiograph showed a round mass 6 x 7 cm in diameter in the right lower lung field (Figure 1). The computed tomographic scan of the chest, enhanced through intravenous injection of contrast media, showed that the mass originated from the right lower pulmonary artery. Behçet disease with pulmonary artery aneurysm was investigated on the basis of the aforementioned findings.
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Figure 1. The posteroanterior chest radiograph showed a round mass 6 x 7 cm in diameter in the right lower lung fields.
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Seven days after admission, the patient had a high fever with sudden hemoptysis of about 30 mL. The follow-up chest radiograph showed the lung mass had increased in size since admission.A magnetic resonance imaging study confirmed a ruptured aneurysm of the right lower lobar pulmonary artery, indicating a pulmonary artery pseudoaneurysm (Figure 2). A lobectomy of the right lower lobe was performed, and a cystic mass containing blood and measuring 4 x 4 x 4 cm in size was found in the superior segment of the lobe. The patient's postoperative course was uneventful. The pathologic findings revealed that the cystic lesion was a ruptured aneurysm of the large pulmonary artery. Elastic staining of this involved pulmonary artery showed that a portion of the wall was destroyed and replaced by inflamed fibrous tissue. The cellular infiltrate of the vessel wall was found to be predominantly composed of lymphocytes, macrophages, neutrophils, and eosinophils. Periadventitial fibrosis was predominant, and an organized thrombus with recanalization of a branch of the pulmonary artery was also found. Vasculitis in this case involved the large pulmonary artery only because vessels of different types and sizes were not affected. A pulmonary artery bronchial fistula formed because of the spread of inflammation and necrosis from the fibrous wall of an elastic pulmonary artery aneurysm into an adjacent bronchus (Figure 3). Seven days postoperatively, the chest tube was removed. During the postoperative course, skin lesions developed as a result of trauma, such as venipuncture and arterial puncture. The patient was administered a course of corticosteroids, and no further hemoptysis and skin lesions occurred in the 20 months after the lobectomy.
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Figure 2. Chest magnetic resonance imaging showing the ruptured aneurysm with the lesion that was interpreted as representing pulmonary artery pseudoaneurysm.
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Figure 3. Spread of inflammation and necrosis from the fibrous wall of an elastic pulmonary artery aneurysm into an adjacent bronchus leads to the formation of a pulmonary artery bronchial fistula. (Elastic staining, original magnification 12.5x.)
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Discussion
The vascular system is affected in 25% of patients with Behçet disease, with specifically pulmonary manifestations occurring in between only 1% and 7% of patients.1 Pulmonary artery aneurysms, in particular, have a poor prognosis and are one of the leading causes of patient death in Behçet disease. Unfortunately, no prospective studies have examined the true prevalence of the pulmonary manifestations of the disease. Aneurysms of pulmonary arteries are very rare. Aortic manifestations are the most common vascular involvement, and pseudoaneurysms in this area have been reported.6,7 Pulmonary artery pseudoaneurysms have not been reported in the literature until our case study. Generally, pulmonary artery pseudoaneurysms occur as a result of erosion of the artery by mycotic aneurysms derived from right-side bacterial endocarditis or infected thrombi from peripheral veins; trauma and chronic pulmonary hypertension, particularly that associated with congenital heart disease, in which a left-to-right shunt develops; and septic emboli. Behçet disease should be included in the listing of diseases causing aneurysms of the large pulmonary arteries. Important complications of pulmonary angitis in Behçet disease include vascular thromboses, pulmonary infarction, bronchial erosion by pulmonary arteries, and pulmonary artery aneurysm.1,2,8 Pulmonary vasculitis in Behçet disease is a unique clinical and pathologic picture.1-4,8 Pulmonary artery aneurysms are located mainly in the right lower lobar arteries, followed by the right and left main pulmonary arteries.8 The pulmonary manifestations appear, on average, 3.6 years after the first extrapulmonary manifestation. Hemoptysis is the major pulmonary manifestation and is reported to occur in 77% of reported cases. Hemoptysis might result from the acute inflammatory destruction of elastic and muscular pulmonary arteries, the rupture of pulmonary artery aneurysms, pulmonary infarcts, erosion of pulmonary artery aneurysm into adjacent bronchi, and finally, the extensive inflammatory disruption of alveolar capillaries.1-5,8 In our case hemoptysis resulted from the erosion of the pulmonary artery pseudoaneurysm into adjacent bronchi. One characteristic of Behçet disease is that vasculitis can occur in different types of pulmonary vessels and might involve large and small vessels simultaneously or separately in the course of the disease.
Chest x-ray films showed abnormal findings in 90% of the cases. Helical computed tomography is currently the method of choice for confirming the diagnosis because it provides an excellent vascular image, requiring only a small quantity of contrast material. Magnetic resonance imaging is also helpful in the diagnosis of pulmonary artery aneurysms and pseudoaneurysms.
Currently, the mainstay of treatment is immunosuppressants, especially when introduced in the early stages before irreversible damage to the arterial wall develops. Anticoagulant therapy holds potential hazards to patients with aneurysmal dilatation of the pulmonary blood vessels. When the disease is localized to one area of the lung, surgical excision, followed by medical treatment, is indicated to prevent fatal pulmonary hemorrhage.
In patients with massive hemoptysis, urgent surgical resection might be necessary and after postoperative steroid treatment because perioperative steroid therapy has been suggested to reduce the risk of complications.
In our patient no further hemoptysis occurred 20 months after the lobectomy.
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