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MAGNETIC RESONANCE IMAGING DETECTION OF AORTIC AND PULMONARY ARTERY WALL THICKENING IN THE ACUTE STAGE OF TAKAYASU ARTERITIS Improvement of Clinical and Radiologic Findings After Steroid Therapy KEN TANIGAWA, KATSUMI EGUCHI, YOSHIO KITAMURA, ATSUSHI KAWAKAMI, HIROAKI IDA, SHUNICHI YAMASHITA, NAOFUMI MATSUNAGA, KUNIAKI HAYASHI, and SHIGENOBU NAGATAKI

Objective. Early diagnosis of Takayasu arteritis in the acute stage (prepulseless stage) is extremely difficult. Identification of a useful approach to detecting the initial changes of arteritis is therefore desirable. Methods. Careful clinical examination of a young woman with persistent fever and dry cough revealed faintly audible bruits at the cervical, supraclavicular, and abdominal regions. Aortographic features suggested thickening of the wall of the descending thoracic aorta. Magnetic resonance imaging (MRI) of this area was diagnostic. Results. MRI demonstrated involvement of the ascending aorta and right main pulmonary artery. From the First Department of Internal Medicine, the Department of Radiology, and the Department of Cell Physiology, Atomic Disease Institute, Nagasaki University School of Medicine, Nagasaki, Japan. Ken Tanigawa, MD: Resident, First Department of Internal Medicine; Katsumi Eguchi, MD: Lecturer, First Department of Internal Medicine; Yoshio Kitamura, MD: Resident, First Department of Internal Medicine; Atsushi Kawakami, MD: Postgraduate Scholar, First Department of Internal Medicine; Hiroaki Ida, MD: Postgraduate Scholar. First Department of Internal Medicine; Shunichi Yamashita, MD: Professor, Department of Cell Physiology, Atomic Disease Institute; Naofumi Matsunaga, MD: Associate Professor, Department of Radiology; Kuniaki Hayashi, MD: Professor, Department of Radiology; Shigenobu Nagataki, MD: Professor, First Department of Internal Medicine. Address reprint requests to Shigenobu Nagataki, MD, First Department of Internal Medicine, Nagasaki University School of Medicine. 7-1 Sakamoto-machi, Nagasaki City, Nagasaki 852, Japan. Submitted for publication March 21, 1991; accepted in revised form December 10, 1991. Arthritis and Rheumatism, Vol. 35, No. 4 (April 1992)

Steroid therapy (prednisolone 60 mg/day) induced dramatic clinical and radiologic improvement in 2 months. Conclusion. This is the first report of MRIdocumented reduction in the thickness of the walls of both the aorta and the pulmonary artery following steroid therapy.

Takayasu arteritis is a chronic inflammatory arteriopathy of unknown cause affecting the aorta, its major branches, and the pulmonary artery ( I ) . During the acute (prepulseless) stage, the manifestations are fever, night sweats, arthralgia, myalgia, cough, hemoptysis, pleurisy, pleural effusion, anemia, and persistent elevation of the erythrocyte sedimentation rate (ESR) (2-6). The late, o r occlusive, stage is dominated by the clinical features originally described as typical manifestations of aortic arch syndrome (1,4,7). Diagnosis of Takayasu artentis during the acute stage may be difficult and can easily be overlooked (1-3,5,8,9). Early diagnosis, however, is very important because the time at which treatment is initiated can affect the prognosis ( I ,2). The significant finding of the acute stage in Takayasu arteritis is reported to be aortic wall thickening (6,lO). Although aortography and computed tomography (CT) can demonstrate the important feature of aortic wall thickening, they do not detect early involvement of the pulmonary artery ( I I ) . We describe here a patient with Takayasu arteritis whose pulmonary artery involvement was diagnosed by magnetic resonance imaging (MRI). Steroid

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Figure 1. Coronal magnetic resonance images (spin-echo 7.(0/20) of a 21-year-old Japanese woman with early, acute Takayasu arteritis. A, There is thickening of the right pulmonary artery (long thin arrows) and a small amount of pericardial effusion (short thick arrows). B, The ascending aorta (arrows).

therapy resulted in a dramatic improvement of clinical and radiologic abnormalities in this patient with early, acute Takayasu arteritis.

CASE REPORT The patient, a 21-year-old Japanese woman, was first admitted to our hospital because of a persistent fever of 39.0"C and dry cough of 2 months duration. The family medical history contributed no diagnostic information. On physical examination, the patient was weak and febrile (temperature >39"C). Her blood pressure was 110/70 mm Hg o n both sides. Bruits were faintly audible at the cervical, supraclavicular, and abdominal regions. Laboratory investigations showed moderate leukocytosis ( I I ,700/mm3), anemia (hemoglobin 10.1 gm/liter), normal platelet count (272,000/mm3), elevated ESR (Westergren) (1 17 mm/hour), elevated C-reactive protein level (31.5 mg/ml), and elevated fibrinogen value (540.5 mg/dl, normal 168.3-328.8). Normal or negative results were found for other serologic tests.

Thoracic and abdominal aortography showed neither stenosis nor dilation, but demonstrated thickening of the wall of the descending thoracic aorta. Digital subtraction angiography of the pulmonary artery and pulmonary perfusion scintigraphy demonstrated no occlusive changes. CT scan showed aortic wall thickening at the levels of the aortic arch and the lower portion of the descending thoracic aorta. MRI revealed that not only were the walls of the aortic arch, the ascending aorta, and the descending thoracic aorta thickened, but also the wall of the right main pulmonary artery was markedly thickened (Figures 1A and B). A small amount of pericardial and pleural effusion was also noted (Figure IA). Takayasu arteritis, acute stage, was diagnosed, and treatment with prednisolone at a dosage of 60 mg/day was begun. Two days after the beginning of steroid therapy, the patient's temperature returned to normal. One month after initiation of treatment, laboratory findings, including the ESR, were normal, and the dry cough had resolved. Followup C T was performed after 2 months of steroid therapy, and showed a reduction in the aortic wall thickening. This finding was confirmed by MRI. The MRI also revealed a

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Figure 2. Coronal magnetic resonance images (spin-echo 750/20) of a 21-year-old Japanese woman with early, acute Takayasu arteritis. 2 months after the initiation of prednisolone therapy (60mg/day). A and B, There is decreased thickening of the arterial walls and resolution of the pericardial effusion.

resolution of the wall thickening of the aortic arch, the ascending aorta, the descending thoracic aorta, and the right main pulmonary artery, as well as a resolution of the pericardial effusion (Figures 2A and B). Thoracic and abdominal aortograms were obtained after 4 months of steroid therapy, and showed decreased aortic wall thickening compared with the previous aortogram. The prednisolone dosage was gradually reduced to a maintenance dosage of 25 mg/day. The ESR has decreased to 10 mmhour, and the patient has experienced complete resolution of her symptoms.

DISCUSSION The diagnosis of Takayasu arteritis in its acute stage is extremely difficult because the clinical features are similar to those of various other diseases (1-3, 5,8,9). Aortography, which shows irregular vessel walls, stenosis, poststenotic dilation, aneurysm formation, and occlusion and collateral circulation (1,4,6), has been used to make the diagnosis. However, these features are also present in the chronic or recurrent stage of the disease. It would be desirable to be able to

diagnose Takayasu arteritis in the early, acute stage, when the inflammatory process involves all layers of the artery, predominantly the media. Pathologic studies reveal granulomatous or diffuse productive inflammation in the media and adventitia by way of the vasa vasorum. Thickening of the intima occurs at the site corresponding to changes in the medial and adventitial layers of larger arteries (12). Periaortic inflammation results in thickening of the arterial wall (13). On aortograms, the thickness of the wall of the descending thoracic aorta is measured as the distance between the intraluminal contrast medium and the air in the lung (lo), but the thickness of the wall of the pulmonary artery is difficult to measure on angiograms. Bond et a1 (14) reported their use of carotid duplex sonography in the demonstration of Takayasu arteritis. While high-resolution ultrasound is less expensive than MRI and CT and can show wall thickening of the common carotid (a usual site of involvement), as well as vascular changes of the abdominal aorta, the thoracic aorta and pulmonary artery cannot be visualized by ultrasound. Recently, there have been reports concerning the diagnostic value of MRI in vascular diseases

MRI OF ARTERIES IN TAKAYASU ARTERITIS (15-18). Miller et al (17) concluded that, compared with angiography, MRI is inadequate for use as a screening tool or for detailed evaluation of arterial diseases other than those affecting the aorta, except in highly selected cases. Most of the major arteries of the thorax and abdomen do not lie in a single plane throughout their course. Although MRI might miss the area of major pathology in the vessels of the proximal arch, thickening of the walls of the aorta and proximal pulmonary artery are better visualized with multisectional scanning by MRI. Indeed, the wall thickening of the vertically positioned aorta can best be seen on axial images, whether by CT or by MRI, and wall thickening of the horizontal portion of the right pulmonary artery would best be evaluated with coronal and sagittal MRI. Followup MRI studies of the patient described here revealed significant reduction of the wall thickening in the aorta and pulmonary artery with steroid therapy. This feature indicates a decrease in active inflammation in and around the aortic wall (Figures 2A and B). Resolution of the pericardial effusion was also demonstrated by MRI. Hayashi et a1 (10) reported the first case of Takayasu arteritis in which reduction of the aortic wall thickening following steroid therapy was documented by CT scans. Although the pulmonary artery is frequently involved in Takayasu arteritis (6,11,19-21), the pulmonary artery lesions found during radiologic examination in patients who apparently have the disease are generally advanced and occlusive. In the acute stage, however, the subtle wall thickening of the proximal portion of the pulmonary artery, without occlusive changes, is difficult to visualize by angiogram or CT scan. MRI provides the advantages of direct imaging in the axial, sagittal, and coronal planes, with good spatial resolution, and because of signal void in the arterial lumen, use of the spin-echo method distinguishes the arterial lumen from its wall without the use of contrast medium. An image section perpendicular to the vessel of interest is best suited for assessing wall thickness by MRI. Although wall thickness is better appreciated on images taken perpendicular to the vessel, the right pulmonary artery is likely to show some partial volume effects because of its oblique course. This does not appear to limit the use of the technique as long as repeat images are carefully acquired at the same anatomic levels and at similar projections. The absence of stenotic or occlusive changes in the aorta, its branches, and the pulmonary artery on

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angiograms does not exclude the possibility of acutestage Takayasu arteritis. Attention should therefore be focused on vascular wall thickening, which can be visualized by CT scan and/or MRI. Furthermore, dramatic improvement in clinical findings and reversal of the active inflammatory process in the aorta and pulmonary artery can be expected with early institution of steroid treatment, which might also prevent future occlusive changes of Takayasu arteritis. We have presented here the first documented case in which an acute inflammatory process of the aorta and the pulmonary artery and the subsequent changes were successfully depicted by spin-echo techniques on MRI.

ACKNOWLEDGMENTS We thank Chikako Tsuruta and Yumi Takahara for their excellent secretarial assistance.

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16. Glazer HS, Gutierrez FR, Levitt RG, Lee JKT, Murphy WA: The thoracic aorta studied by MR imaging. Radiology 157~149-155, 1985 17. Miller DL, Reinig JW, Volkman DJ: Vascular imaging with MRI: inadequacy in Takayasu’s arteritis compared with angiography. AJR Am J Roentgenol 146:949-954, 1986 18. Mayo J , Culham JAG: Magnetic resonance imaging in pediatric vascular disease. J Can Assoc Radiol 38: 165169, 1987 19. Suzuki Y, Konishi K, Hisada K: Radioisotope lung scanning in Takayasu’s arteritis. Radiology 109: 133-136, 1973 20. Lupi HE, SBnchez G, Horwitz S, Gutierrez E: Pulmonary artery involvement in Takayasu’s artentis. Chest 67:69-74, 1975 21. Matsunaga N, Hayashi K, Aikawa H, Iwano M, Matsuoka Y, Hombo Z, Fukushima T, Maeda H: Digital subtraction angiography in Takayasu’s arteritis. Acta Radiol 28:247-252, 1987

Magnetic resonance imaging detection of aortic and pulmonary artery wall thickening in the acute stage of Takayasu arteritis. Improvement of clinical and radiologic findings after steroid therapy.

Early diagnosis of Takayasu arteritis in the acute stage (prepulseless stage) is extremely difficult. Identification of a useful approach to detecting...
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