DOI: 10.1148/rg.243035091
RadioGraphics 2004;24:801-808
© RSNA, 2004
Thoracic Manifestations of Behçet Disease at CT1
Nurith Hiller, MD,
Sivan Lieberman, MD,
Tova Chajek-Shaul, MD,
Jacob Bar-Ziv, MD and
Dorith Shaham, MD
1 From the Department of Radiology, Hadassah University Hospital, Ein-Kerem, Jerusalem, Israel 91120 (N.H., S.L., J.B.Z., D.S.); and the Department of Internal Medicine, Hadassah Mount Scopus University Hospital, Jerusalem, Israel (T.C.S.). Presented as an education exhibit at the 2002 RSNA scientific assembly. Received April 1, 2003; revision requested May 16 and received June 27; accepted June 27. All authors have no financial relationships to disclose. Address correspondence to D.S. (e-mail: dshaham@hadassah.org.il).
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Abstract
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Behçet disease is a multisystemic and chronic inflammatory disorder of unknown cause that is characterized by recurrent oral and genital ulcerations, ocular manifestations, and additional clinical manifestations in multiple organ systems. Behçet disease involving the chest can manifest as a wide spectrum of abnormalities. Although conventional chest radiography is commonly used for initial assessment, spiral computed tomography can demonstrate the entire spectrum of thoracic manifestations of Behçet disease, including abnormalities of the vessel lumen and wall, perivascular tissues, lung parenchyma, pleura, and mediastinal structures. Aneurysms of the pulmonary arteries, with or without thrombosis, are a typical manifestation of Behçet disease. Other manifestations include thrombosis, vasculitis, hemorrhage, infarction, and inflammation. Familiarity with these manifestations can be useful in the diagnosis of Behçet disease, helping to determine the cause of symptoms in patients who present with hemoptysis and guide the choice of appropriate therapy.
© RSNA, 2004
Index Terms: Behçet disease, 92.629, 94.629 • Lung, diseases, 60.629 • Pulmonary arteries, diseases, 944.629 • Pulmonary arteries, thrombosis, 944.751 • Thorax, CT, 94.1211 • Thorax, diseases, 94.629
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LEARNING OBJECTIVES
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After reading this article and taking the test, the reader will be able to:
- Describe the clinical and pathologic features of Behçet disease.
- Recognize the CT manifestations of Behçet disease of the chest.
- Discuss the unique features of pulmonary arterial thrombosis in Behçet disease.
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Introduction
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Behçet disease is a multisystemic and chronic inflammatory disorder whose cause is unknown. The clinical triad of oral and genital ulcerations and ocular manifestations was originally described by the Turkish dermatologist Hulusi Behçet in 1937 (1). Additional clinical manifestations in other locations (skin, joints, gastrointestinal tract, genitourinary tract, central nervous system, cardiovascular system, lung) were described later (2). Vascular complications develop in about 20%–40% of patients with Behçet disease (3,4). Pulmonary involvement is relatively infrequent, having been reported in 1%–10% of patients (2,3,5). The underlying pathologic process in Behçet disease is vasculitis and perivascular inflammatory infiltrates affecting vessels of differing sizes in various organs (3,6–11).
The mean age at which Behçet disease occurs is 20–30 years (6,12). The disease is most prevalent in the Mediterranean region, Middle East, and Far East, and males are affected 2–5 times more often than females. The highest prevalence (80–370 cases per 100,000 persons) was reported in Turkey (2). The prevalence of Behçet disease is now increasing in other parts of the world, especially Europe and America, due to migration.
Currently, computed tomography (CT) is the preferred imaging modality for depicting the thoracic manifestations of Behçet disease. In this article, we review the clinical diagnosis of Behçet disease and imaging considerations in this setting. We also discuss and illustrate the spectrum of CT findings in Behçet disease of the thorax, including vascular, pulmonary parenchymal, pleural, and mediastinal abnormalities.
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Clinical Diagnosis of Behçet Disease
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According to the diagnostic criteria of the International Study Group for Behçet Disease (13), the diagnosis is based on the presence of recurrent oral ulcerations, along with two of the following criteria: (a) recurrent genital ulcerations, (b) eye lesions, including uveitis and retinal vasculitis, (c) skin lesions (folliculitis, erythema nodosum), and (d) positive skin pathergy test (pustule formation 24–48 hours following skin prick) (13). The Table shows the frequency with which various clinical manifestations of Behçet disease occur.
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Imaging Considerations
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Conventional chest radiography is commonly used for initial assessment of pulmonary signs and symptoms of Behçet disease, for follow-up, and for assessment of response to therapy. The pulmonary parenchymal changes are nonspecific and appear as focal and diffuse areas of increased opacity (3,14). Chest radiographs are also useful for detection of hilar enlargement, which may be due to pulmonary artery aneurysms (14,15), and of mediastinal widening, which may be an ominous sign of an aneurysm developing in the thoracic aorta (6).
Angiography and venography should not be performed in patients with Behçet disease due to increased risk for (a) aneurysm formation at the arterial puncture site and (b) venous thrombosis after contrast material injection (4,12). In addition, aneurysms may not be depicted at angiography if they are completely thrombosed (16,17).
Magnetic resonance (MR) angiography can be used for imaging of pulmonary artery aneurysms, thrombosis within these aneurysms, venous occlusions, and the development of collateral vessels. Although MR angiography is useful for imaging of vascular complications involving the superior vena cava (SVC), aorta, and pulmonary arteries, it is of limited value in imaging the lung parenchyma (18).
Spiral CT is useful in demonstrating the entire spectrum of thoracic manifestations of Behçet disease. Spiral CT is noninvasive and provides excellent delineation of the vessel lumen and wall and perivascular tissues as well as detailed information concerning the lung parenchyma, pleura, and mediastinal structures. CT angiography can be performed with only a small quantity of contrast material and may be used as an alternative to venography and angiography.
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Thoracic Manifestations of Behçet Disease
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Behçet disease has a wide spectrum of thoracic manifestations involving a variety of anatomic structures within the chest (2,6).
Thoracic Vessels
Although vascular involvement is seen in only 25% of patients, it is the most common cause of mortality in Behçet disease (4,6,12). Behçet disease associated with lesions in the large vessels is referred to as "vasculo-Behçet disease" and includes venous or arterial occlusion and aneurysm formation (11). Systemic arterial manifestations of Behçet disease are infrequent compared with venous involvement, accounting for only 12% of vascular complications (2,19). An arterial lesion may develop in the aorta or the pulmonary artery and its major branches and manifests as an aneurysm in 65% of patients and as an occlusion in 35% (4,14).
Behçet disease is the most common cause of pulmonary artery aneurysm (Figs 1, 2) (15). The underlying pathophysiologic process is inflammation of the vasa vasorum of the tunica media, which causes destruction of the elastic fibers of the media and dilatation of the vessel lumen. Thickening of the vessel wall is caused by inflammation and infiltration by lymphocytes, plasma cells, and neutrophils (3). Thrombosis of the pulmonary arteries in Behçet disease is usually in situ thrombosis (Fig 3a, 3b). Although deep vein thrombosis is common in Behçet disease, pulmonary embolism is rare because the thrombi in the inflamed veins of the lower extremities are strongly adherent (20). Hemoptysis is the most common presenting symptom and is one of the leading causes of death. Possible causes for hemoptysis include rupture of an aneurysm with erosion into a bronchus and thrombosis of pulmonary vessels (2). Aneurysm formation in the pulmonary arteries indicates a poor prognosis: 30% of patients with this condition will die within 2 years (3,20,21). Immunosuppressive therapy can cause regression or complete disappearance of pulmonary artery aneurysms, as shown by Tunaci et al (22) (Fig 3c).
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Figure 1. Pulmonary artery aneurysms with mural thrombi in a 13-year-old girl who presented with hemoptysis. Semicoronal contrast material-enhanced chest CT scan (mediastinal window) shows extensive bilateral pulmonary artery aneurysms and mural thrombi (arrows).
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Figure 2. Pulmonary artery aneurysm in a 37-year-old patient with hemoptysis. Axial contrast-enhanced chest CT scan (mediastinal window) demonstrates an aneurysm of the right main pulmonary artery (arrows). Note the apparent wall thickening due to vasculitis or thrombosis.
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Figure 3a. Pulmonary artery aneurysms in a 20-year-old patient with hemoptysis. (a) Contrast-enhanced chest CT scan (mediastinal window) shows vasculitis and thrombosis in multiple peripheral pulmonary arteries. Note the large aneurysm on the left side and the smaller ones on the right side (arrows). (b) Contrast-enhanced chest CT scan (lung window) again shows the aneurysms (thin arrows). Note also the small, peripheral round lesion in the lingula (thick arrow), a finding that is probably due to vasculitis or infarction. (c) CT scan obtained 2 years later following therapy shows complete resolution of the pulmonary artery aneurysms.
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Figure 3b. Pulmonary artery aneurysms in a 20-year-old patient with hemoptysis. (a) Contrast-enhanced chest CT scan (mediastinal window) shows vasculitis and thrombosis in multiple peripheral pulmonary arteries. Note the large aneurysm on the left side and the smaller ones on the right side (arrows). (b) Contrast-enhanced chest CT scan (lung window) again shows the aneurysms (thin arrows). Note also the small, peripheral round lesion in the lingula (thick arrow), a finding that is probably due to vasculitis or infarction. (c) CT scan obtained 2 years later following therapy shows complete resolution of the pulmonary artery aneurysms.
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Figure 3c. Pulmonary artery aneurysms in a 20-year-old patient with hemoptysis. (a) Contrast-enhanced chest CT scan (mediastinal window) shows vasculitis and thrombosis in multiple peripheral pulmonary arteries. Note the large aneurysm on the left side and the smaller ones on the right side (arrows). (b) Contrast-enhanced chest CT scan (lung window) again shows the aneurysms (thin arrows). Note also the small, peripheral round lesion in the lingula (thick arrow), a finding that is probably due to vasculitis or infarction. (c) CT scan obtained 2 years later following therapy shows complete resolution of the pulmonary artery aneurysms.
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Thickening of the vessel wall due to vasculitis can be seen in the aorta (Fig 4) and SVC (Fig 5).
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Figure 5. SVC wall thickening in a 40-year-old patient who presented with SVC obstruction. Contrast-enhanced chest CT scan (mediastinal window) demonstrates marked thickening of the SVC wall due to vasculitis (arrows).
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SVC thrombosis is not uncommon in Behçet disease (Fig 6) (23) and is often accompanied by thrombosis of other mediastinal veins.
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Figure 6. SVC thrombosis in a 62-year-old patient with a 25-year history of SVC occlusion. Contrast-enhanced chest CT scan (mediastinal window) demonstrates a narrow and fibrotic SVC (thick arrow) and extensive collateral vessels in the mediastinum and chest wall (thin arrows).
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Pulmonary Parenchyma
The pulmonary manifestations of Behçet disease include pulmonary infarcts, pulmonary hemorrhage, atelectasis, cryptogenic organizing pneumonia, eosinophilic pneumonia, recurrent pneumonia, bronchitis, fibrosis, and emphysema. Pulmonary vasculitis and thrombosis of pulmonary vessels result in infarction, hemorrhage, and focal atelectasis. The most common parenchymal lesions are subpleural alveolar infiltrates and wedge-shaped or ill-defined rounded areas of increased opacity, which represent focal vasculitis with hemorrhage, infarction, and inflammation (Fig 7) (6).
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Figure 7a. Vasculitis with hemorrhage and infarction. (a, b) Chest CT scans (lung window) obtained at different levels of the right lower lobe demonstrate focal areas of high attenuation in the periphery of the lobe (arrows), findings that represent vasculitis with hemorrhage and infarction. (c) Chest CT scan (lung window) obtained in a different patient demonstrates similar findings in the right upper lobe (arrows).
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Figure 7b. Vasculitis with hemorrhage and infarction. (a, b) Chest CT scans (lung window) obtained at different levels of the right lower lobe demonstrate focal areas of high attenuation in the periphery of the lobe (arrows), findings that represent vasculitis with hemorrhage and infarction. (c) Chest CT scan (lung window) obtained in a different patient demonstrates similar findings in the right upper lobe (arrows).
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Figure 7c. Vasculitis with hemorrhage and infarction. (a, b) Chest CT scans (lung window) obtained at different levels of the right lower lobe demonstrate focal areas of high attenuation in the periphery of the lobe (arrows), findings that represent vasculitis with hemorrhage and infarction. (c) Chest CT scan (lung window) obtained in a different patient demonstrates similar findings in the right upper lobe (arrows).
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Diffuse airspace areas of increased opacity with rapid resolution, especially in patients with hemoptysis, often represent pulmonary hemorrhage (Fig 8). Airspace consolidation may also be due to organizing pneumonia, which may accompany other collagen vascular diseases such as rheumatoid arthritis and systemic lupus erythematosis(2). Airspace consolidation can also be a manifestation of infectious pneumonia, which might have a recurrent course (Fig 9) (7). Pneumonias in Behçet disease can be the result of inflammation of pulmonary parenchymal vessels or may occur secondary to immunosuppressive therapy (7,24).
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Figure 8. Pulmonary hemorrhage in a 28-year-old patient with massive hemoptysis. Chest CT scan (lung window) shows patchy consolidation in the left lower lobe (arrow), a finding that is consistent with pulmonary hemorrhage.
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Damaged lung tissue can be replaced by fibrosis or emphysema. Focal or diffuse air trapping may result from airway obstruction due to small airway inflammation and fibrosis (Figs 10, 11) (25,26).
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Figure 10. Vasculitis of the pleura. Chest CT scan (lung window) demonstrates bilateral areas of hypoattenuation in the upper lobes due to either hypoperfusion or air trapping. Note also the peripheral focal lesion in the left upper lobe (black arrow) and multiple tiny pleural nodules in both lobes (white arrows), the latter findings being consistent with vasculitis of the pleura.
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Figure 11. Small airway disease. Chest CT scan (lung window) shows small airway disease with a "tree-in-bud" appearance and mild bronchiectatic changes (thin arrow) in the right lower lobe. Note also the linear scarring (thick arrow).
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Pleura
Vasculitis of the pleura may result in the formation of pleural nodules, which are often difficult to differentiate from parenchymal subpleural lesions (Fig 10). Pleural effusion may be attributed to pulmonary infarction, vasculitis of the pleura, or SVC thrombosis (Fig 12) (6).
Mediastinum and Heart
Mediastinal lymphadenopathy may be seen and is probably a reaction to an inflammatory process in the chest. An inflammatory process in the mediastinum may manifest as a mediastinal mass (Fig 13).
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Figure 13. Inflammatory mediastinal mass in a 36-year-old patient. Contrast-enhanced chest CT scan (mediastinal window) demonstrates a diffuse mediastinal process surrounding the mediastinal vessels (arrows). Analysis of the needle biopsy specimen revealed benign lymphocytic infiltration.
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Intracardiac thrombosis has been described, with the right side of the heart being the most frequent site of involvement (27,28).
Pericardial effusion may result from vasculitis of the pericardium or SVC thrombosis (Fig 14).
Aneurysms of the aortic arch and subclavian and coronary arteries have also been described (Fig 15) (2,6).
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Figure 15. Coronary artery aneurysm in a 20-year-old patient. Contrast-enhanced chest CT scan (mediastinal window) shows a calcified aneurysm of the left anterior descending coronary artery (arrow).
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Conclusions
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Behçet disease involving the chest can manifest as a wide spectrum of abnormalities. Aneurysms of the pulmonary arteries with or without thrombosis are a typical manifestation of Behçet disease. Involvement of the SVC and aorta may occur, and pulmonary findings include pulmonary hemorrhage and atelectasis, fibrosis, and air trapping. The mediastinum and pleura may also be involved. Knowledge of these various manifestations can be useful in diagnosing Behçet disease, documenting the cause of symptoms in patients who present with hemoptysis, and initiating appropriate therapy.
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Footnotes
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Abbreviation: SVC = superior vena cava
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References
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Abstract
LEARNING OBJECTIVES
