Systemic artery--pulmonary artery communication in Takayasu's arteritis.

Four cases of Takayasu's arteritis in which systemic artery-pulmonary artery communication is demonstrated on thoracic aortography are presented. Pulmonary arterial involvement in Takayasu's arteritis seems to be more frequent than generally appreciated (12% in the present series). Demonstration of the communication in the absence of evidence of other causes of a shunt is strongly suggestive of pulmonary artery involvement. It is not necessary to perform pulmonary angiography to confirm the involvement unless clinically indicated. It is also stressed that the presence of pulmonary artery involvement is useful to differentiate Takayasy's arteritis from arteriosclerosis.     

Case report: therapeutic bronchial artery embolization in a case of Takayasu's arteritis.

The pulmonary arteries are frequently involved in Takayasu's arteritis with a reported incidence of 41-100% in affected individuals. Patients are usually asymptomatic, however, often despite extensive pulmonary involvement. We describe a patient with Takayasu's arteritis who presented with haemoptysis caused by bronchial artery hypertrophy secondary to occlusive pulmonary arterial disease, who was treated by bronchial artery embolization. This is, to our knowledge, the first reported case of haemoptysis secondary to Takayasu's arteritis successfully treated by embolization.     

Initial isolated Takayasu's arteritis of the right pulmonary artery: MR appearance

Takayasu's arteritis involves the pulmonary artery tree in more than 50% of the cases. Initial isolated involvement of the pulmonary artery by Takayasu's arteritis, however, is very rare. We report the case of a 34-year-old white woman who presented a clinical and radiographic pattern that mimicked an acute pulmonary embolism with pulmonary infarction. Pulmonary angiography showed stenosis lesions and occlusion of the right pulmonary artery tree. Magnetic resonance imaging demonstrated thickening of the pulmonary artery wall leading to the correct diagnosis Correspondence to: G. Ferretti     

Pulmonary artery disease in Takayasu's arteritis: angiographic findings.

One hundred sixteen arteriographic examinations in 98 patients with Takayasu's arteritis were studied retrospectively to evaluate the extent of aortic and pulmonary disease. Stenosis was the most frequent finding in the aorta and its branches, but occlusion, dilatation, and aneurysms were also seen. Adventitial vascular structures, consistent with dilated vasa vasorum, were observed in five patients (5%) and systemic artery-pulmonary artery communication was seen in six (6%). Twenty-one (70%) of 30 patients who had pulmonary arteriography were shown to have pulmonary artery involvement. Upper lobe pulmonary arterial branches showed abnormalities most frequently; segmental branches, followed by subsegmental branches, were most often involved. The frequency of abnormal findings on pulmonary arteriograms correlated with the number of involved brachiocephalic vessels. Stenotic lesions in the aorta and its branches progressed in five (45%) of 11 patients, and those in the pulmonary artery progressed in one (33%) of three cases. Takayasu's arteritis characteristically involves the systemic and pulmonary arteries, and the extent of arteritis in the major branches of the aortic arch appears to correlate with pulmonary arterial involvement. Both thoracic and abdominal aortographic studies and pulmonary arteriography are necessary to properly diagnose the extent of the disease, and angiography should be repeated for appropriate patient follow-up.     

大动脉炎累及肺动脉肺通气/灌注显像一例

笔者报道了一例大动脉炎累及肺动脉肺通气/灌注显像的病例。从临床症状、体征、实验室检查、影像学检查等方面分析该病例特点。肺通气/灌注显像示右肺全肺、左肺上叶尖后段、左肺下叶背段、左肺外基底段的肺通气、肺灌注显像不匹配。仅从肺通气/灌注显像结果来看,易误诊为肺栓塞,但结合患者病史、检查结果及相关文献,综合分析考虑为大动脉炎累及肺动脉。通过文献复习加深了对大动脉炎的认识。对于大动脉炎累及肺动脉患者,肺动脉造影检查为有创性,而肺通气/灌注显像为无创性,且诊断准确率也较高,不失为一种较好的诊断及疗效评估的手段,提示临床医师尽早采取积极的对症治疗措施,改善患者预后。     

Delayed contrast-enhanced MRI of the aortic wall in Takayasu's arteritis: initial experience

OBJECTIVE: Delayed contrast-enhanced MRI is increasingly being used for cardiac viability imaging. Takayasu's arteritis is a rare inflammatory disorder of unknown cause that affects the aorta, its major branches, and the pulmonary artery; it is characterized by inflammation and fibrosis in the arterial wall. We report our initial experience with seven patients (six women, one man; age range, 25-62 years) with delayed (20 min) gadolinium-enhanced MRI (inversion recovery prepared gated fast gradient-echo pulse sequence) in patients with known Takayasu's arteritis. CONCLUSION: Patients with Takayasu's arteritis (particularly those with abnormal laboratory values) have evidence of delayed hyper-enhancement on delayed contrast-enhanced MRI. Thus, delayed contrast-enhanced MRI might be a useful technique to identify inflammation in arterial wall.     

Pulmonary vascular disease

This review article discusses three topics related to pulmonary vascular disease: 1) pulmonary vascular changes associated with portal hypertension, 2) ANCA-associated pulmonary vasculitis, and 3) Takayasu's arteritis. Hepatopulmonary syndrome and pulmonary hypertension have recently been reported as pulmonary vascular changes accompanied with portal hypertension. Endogenous vasoactive agents that reach the pulmonary circulation through porto-systemic shunt vessels are thought to contribute to these vascular changes. In ANCA-associated vasculitis, hemorrhage, interstitial pneumonitis, and nodular lesions are common manifestations in the lung. In Takayasu's arteritis, CT occasionally demonstrates mosaic attenuation owing to pulmonary arteritis and peripheral reticulolinear changes probably due to thromboembolism.     

Comprehensive analysis of perfusion scintigraphy in Takayasu's arteritis.

One hundred eighty perfusion lung scans of 120 patients with Takayasu's arteritis were retrospectively evaluated. Lung scans were abnormal in 76% of the patients. Lung scans could be classified into four groups, according to the pattern of perfusion defects. Early involvement occurred in the upper lobes, and the middle and lower lobes were involved at a later stage. The data obtained by physiological examination (spirography and arterial blood gas analysis) showed a weak relationship with the severity of perfusion lung scan findings. In addition, repeated lung scans were found to be easy and reliable in the follow-up period of pulmonary artery involvement.     

Aortic aneurysms in patients with Takayasu's arteritis: CT evaluation

OBJECTIVE: The objective of this study was to evaluate the incidence, development, and outcome of aortic aneurysm on CT in a group of patients with Takayasu's arteritis. MATERIALS AND METHODS: We reviewed the files of 31 patients with Takayasu's arteritis between January 1990 and March 1999. All patients were followed up for more than 6 months with CT, and the mean follow-up period was 52.9 months (median, 36.0 months). In all patients, initial CT was performed within 6 months of diagnosis of Takayasu's arteritis. The study group comprised 24 female patients and seven male patients; their ages at the first CT examination ranged from 8 to 72 years (mean, 42. 6 +/- 16.5 years). RESULTS: Seventeen aortic aneurysms were found in 14 (45.2%) of the 31 patients. Patients with severe calcification of the aorta showed significantly lower incidence of aneurysm formation than those without severe calcification of the aorta (p < 0.05). Of the 17 aneurysms, three were not present at the time of initial CT and appeared during the follow-up period. Nine of 17 aneurysms increased in size during the follow-up period. Three of the nine aneurysms rapidly increased in size and ruptured during the follow-up period. In all three of these aneurysms, aortic wall thickening was identified on CT. The remaining six aneurysms slowly increased in size but did not rupture. CONCLUSION: Aortic aneurysm associated with Takayasu's arteritis is not rare. The aorta with little calcification has a greater possibility of aneurysm formation in patients with Takayasu's arteritis. Aortic aneurysms with wall thickening can have fatal consequences.     

The incidence and patterns of pulmonary artery involvement in Takayasu's arteritis

The pulmonary arterial anatomy in 44 patients with Takayasu's arteritis was examined by intravenous digital subtraction angiography (IV-DSA) on an outpatient basis using centrally delivered, small-volume, bolus injections of an ionic, water soluble contrast medium. Diagnostic pulmonary angiograms were obtained in 42 patients (95.4%) without complication. Angiographically evident pulmonary arterial involvement was seen in six patients (14.3%). The pulmonary involvement was not suspected clinically in any patient and the chest radiographs were abnormal in only two patients (33%). The angiographic spectrum of systemic arterial involvement was the same irrespective of the presence or absence of pulmonary arterial involvement. The pulmonary arterial pressures were measured in two patients with abnormal pulmonary angiograms and were found normal. Follow up IV-DSA in one of these patients 16 months after immuno-suppressive therapy showed no change in the pulmonary angiographic picture.     

Takayasu's arteritis: assessment of disease activity with contrast-enhanced MR imaging

OBJECTIVE: The purpose of this study was to evaluate the role of contrast-enhanced MR imaging in the determination of disease activity in patients with Takayasu's arteritis. SUBJECTS AND METHODS: High-resolution contrast-enhanced T1-weighted spinecho MR imaging using small fields of view (14-20 cm) and thin slices (4-5 mm) was performed in 26 patients with Takayasu's arteritis and 16 healthy subjects. The degree of aortic mural enhancement was assessed by measuring signal intensity and by visually estimating it in comparison with that of the myocardium. RESULTS: Contrast-enhanced MR imaging showed more enhancement of thickened aortic wall compared with myocardium, thus suggesting active Takayasu's arteritis on MR imaging in 16 patients. Determination of disease activity using contrast-enhanced MR imaging was concordant with clinical findings in 23 patients (88.5%). Contrast-enhanced MR findings were concordant with laboratory findings in most patients (erythrocyte sedimentation rate in 92.3% [24/26] and C-reactive protein in 84.6% [22/26]). The measured signal intensity of the aortic wall relative to that of myocardium during the early phase of contrast-enhanced MR imaging correlated well with the erythrocyte sedimentation rate (r = 0.78, p < 0.005) and with the C-reactive protein level (r = 0.63, p < 0.005). CONCLUSION: Contrast-enhanced MR imaging provides information about disease activity of Takayasu's arteritis, which may be useful in the diagnosis and treatment of Takayasu's arteritis.     

Radionuclide renography predicts functional changes in patients with renal artery involvement by Takayasu's arteritis

Renovascular hypertension is a major complication of Takayasu's arteritis, which contributes to the high mortality associated with the disease. We studied 5 patients affected by different degrees of Takayasu's arteritis to assess the usefulness of radionuclide renography in evaluating renal perfusion and function, and to predict changes induced by the disease before and after therapeutic interventions. Computer-assisted dynamic renal imaging with Tc-99m diethylenetriaminepentaacetic acid (DPTA) and I-131 orthoiodohippurate (OIH), and renal arteriography were concurrently performed in all patients. Two patients with hemodynamically insignificant renal artery stenosis showed normal perfusion and function by renography. Three patients had significant renal artery stenosis and functional changes on renography. Subsequently, two of these patients had successful therapy (one had bilateral renal artery bypass grafts, and the other had renal artery angioplasty), and both showed functional improvement at renography. Our results demonstrate that radionuclide renography is valuable in the assessment of functional changes induced by Takayasu's arteritis as well as for determining the response to therapeutic interventions.     

Ventilation-perfusion lung scan for the detection of pulmonary involvement in Takayasu's arteritis.

The aim of study was to analyse ventilation and perfusion (V/Q) lung scan findings in a series of Italian patients with Takayasu's arteritis. Eighteen consecutive patients underwent V/Q lung planar scintigraphy and single-photon emission tomography (SPET). Before perfusion scan acquisition was started, a first-pass study with (99m)Tc-macroaggregates of albumin was performed to assess the right ventricular ejection fraction (RVEF). All patients had normal chest X-rays and were symptom free at the time of the investigation. They also underwent echocardiography to evaluate pulmonary artery pressure and in 13 patients respiratory function tests were performed. In four patients, perfusion lung scan was repeated after 1 year. In 10/18 patients (55.5%), 43 unmatched lobar, segmental or subsegmental perfusion defects were found on planar images; ventilation scintigraphy was normal in all cases. On SPET images, 55 defects were found; no defects were found with SPET in the remaining patients who had normal planar images. All patients had normal RVEF and 5/13 patients had mild restrictive-obstructive lung disease. The pulmonary artery pressure was increased in two patients with perfusion defects. In the four patients who had repeat scintigraphy, all defects remained unchanged. The prevalence of lung perfusion abnormalities observed in Italian patients with Takayasu's arteritis is within the range of values reported in other countries, and V/Q planar scintigraphy is sufficient for the screening of patients.     

Nonsurgical treatment of Takayasu's disease

Success of percutaneous transluminal angioplasty (PTA) in disorders other than atherosclerosis has been reported in recent papers. It has been stated, however, that the unique histopathology characteristic of Takayasu's arteritis may prevent a successful dilatation of the stenotic segments. In a recent case, however, PTA was successful in the treatment of bilateral renal artery stenosis secondary to Takayasu's arteritis. Although more time is needed to evaluate the long-term results of this nonsurgical treatment, it is evident that the stenotic lesions in Takayasu's disease are amenable to balloon dilatation. PTA should be attempted in such cases, and surgery should only be performed in case of failure.     

Ventilation-perfusion lung scan for the detection of pulmonary involvement in Takayasu's arteritis

The aim of study was to analyse ventilation and perfusion (V/Q) lung scan findings in a series of Italian patients with Takayasu's arteritis. Eighteen consecutive patients underwent V/Q lung planar scintigraphy and single-photon emission tomography (SPET). Before perfusion scan acquisition was started, a first-pass study with ^99mTc-macroaggregates of albumin was performed to assess the right ventricular ejection fraction (RVEF). All patients had normal chest X-rays and were symptom free at the time of the investigation. They also underwent echocardiography to evaluate pulmonary artery pressure and in 13 patients respiratory function tests were performed. In four patients, perfusion lung scan was repeated after 1 year. In 10/18 patients (55.5%), 43 unmatched lobar, segmental or subsegmental perfusion defects were found on planar images; ventilation scintigraphy was normal in all cases. On SPET images, 55 defects were found; no defects were found with SPET in the remaining patients who had normal planar images. All patients had normal RVEF and 5/13 patients had mild restrictive-obstructive lung disease. The pulmonary artery pressure was increased in two patients with perfusion defects. In the four patients who had repeat scintigraphy, all defects remained unchanged. The prevalence of lung perfusion abnormalities observed in Italian patients with Takayasu's arteritis is within the range of values reported in other countries, and V/Q planar scintigraphy is sufficient for the screening of patients.     

Initial experience with percutaneous transluminal angioplasty in the management of Takayasu's arteritis

We have performed percutaneous transluminal angioplasty (PTA) for 15 arterial stenoses in 11 patients with Takayasu's arteritis. The lesions included tight, proximally located renal artery stenosis (12 stenoses; nine patients), localized abdominal aortic stenosis (two patients) and occluded left common iliac artery (one patient). Clinically successful dilatation was achieved in seven patients (10 stenoses) with renal artery stenosis, in both the patients with abdominal aortic stenosis and in the only patient with an occluded left common iliac artery. No complications related to the procedure were encountered. The follow-up period (n = 7) ranged between 1 and 16 months, mean follow-up period after renal angioplasty was 5 months and after abdominal aortic angioplasty was 12.5 months. Initial success has been maintained in both the patients with abdominal aortic stenoses and in four out of five patients with renal artery stenosis. Percutaneous transluminal angioplasty offers an attractive alternative for the management of stenosing lesions in Takayasu's arteritis with good short term results.     

Coronary artery to pulmonary artery collaterals in nonspecific aortoarteritis involving the pulmonary arteries

We report a patient with nonspecific aortoarteritis (Takayasu's disease) in whom occlusion of the right upper lobe pulmonary artery was associated with collateral flow from the left circumflex coronary artery. Coronary to pulmonary artery collaterals are rare in this disease but have important clinical implication because of their ability to produce coronary steal and myocardial ischemia. Awareness of these pathways is essential for their detection in patients with angina-like symptoms and for differentiation of myocardial ischemia due to direct coronary artery involvement by this disease.     

Therapeutic approach to progressive Takayasu's arteritis

BACKGROUND: Takayasu's arteritis (TA) is a nonspecific autoimmune inflammation manifested with systemic large vessel vasculitis which affects predominantly the aorta and its main branches. CASE REPORT: We presented a 40-year-old woman with a two-year history of the symptoms of compromised circulation in the head, the neck, the upper extremities and the right leg, followed by claudications in the left leg, numbness in the left hand and stenocardia. Aortic arch angyography revealed occlusion of the right subclavian artery stenosis of the right vertebral artery (20-30%) and the right iliac artery. Anti-inflammatory agents had no satisfactory effect and due to the discase progression first aorto-bifemoral bypass grafting, and 10 months later left carotid-axillary bypass grafting were performed which led to the restoration of circulation in the lower extremities and the left arm. CONCLUSION: This case suggested that a surgical therapy should be applied in the cases with progressive Takayasu's arteritis, particularly in the absence of a response to the conventional immunosupressive therapy to prevent eventual fatal complications.     

Imaging findings in Takayasu's arteritis

OBJECTIVE: The objective of our study was to evaluate the clinical usefulness of cross-sectional imaging for establishing the diagnosis of Takayasu's arteritis (TA), an inflammatory vascular disorder that produces arterial stenoses and aneurysms primarily involving the thoracoabdominal aorta and its branches and the pulmonary arteries. CONCLUSION: CT and MRI findings of TA include vascular wall thickening and enhancement early in the disease, and arterial stenoses, occlusions, and aneurysms later in the disease. Cross-sectional imaging is useful for establishing the diagnosis of TA and for showing response to nonsurgical therapy or for planning a surgical intervention.     

Dissection of the abdominal aorta in a child with Takayasu's arteritis

Takayasu's arteritis is a chronic inflammatory disease that primarily involves the aorta and its main branches. Varying degrees of narrowing, occlusion, or dilatation develop in the involved vessel segments. However, dissection of the aorta is quite rare in this disease, and it may develop particularly after angioplasty. We report a very rare case of Takayasu's arteritis with dissection of the abdominal aorta just distal to the origin of the inferior mesenteric artery in a 9-year-old girl. She was treated conservatively with close follow-up. At the end of 1 year's follow-up, the dissection of the aorta did not show progression, and new lesions were not identified. To our knowledge, this patient is the youngest child presented with arterial dissection as the initial manifestation of the disease.