Takayasu disease with prominent pulmonary artery involvement: confusion with pulmonary disease leading to delayed diagnosis

Pulmonary artery involvement as the initial predominant clinical manifestation in Takayasu arteritis (TA) is rare. We describe a young adult female who presented with life-threatening complications of proximal pulmonary arterial involvement of Takayasu arteritis. In our case, atypical presentation of TA with pulmonary symptoms due to pulmonary artery involvement resulted in an erroneous initial diagnosis of sarcoidosis and then tuberculosis. The frequency of such a clinical form could be underestimated given the difficulties involved in its diagnosis and because its features are similar to those of pulmonary disease.     

Takayasu disease with prominent pulmonary artery involvement: confusion with pulmonary disease leading to delayed diagnosis

Abstract

Pulmonary artery involvement as the initial predominant clinical manifestation in Takayasu arteritis (TA) is rare. We describe a young adult female who presented with life-threatening complications of proximal pulmonary arterial involvement of Takayasu arteritis. In our case, atypical presentation of TA with pulmonary symptoms due to pulmonary artery involvement resulted in an erroneous initial diagnosis of sarcoidosis and then tuberculosis. The frequency of such a clinical form could be underestimated given the difficulties involved in its diagnosis and because its features are similar to those of pulmonary disease.     

Takayasu's disease localized exclusively in the pulmonary artery

A 35 year-old woman exhibited a stenotic lesion of the right pulmonary artery with a severe inflammatory syndrome. A diagnosis of Takayasu arteritis was made, although no aortographic abnormality was present. Involvement of pulmonary arteries during the course of Takayasu arteritis is frequent, but a localisation of the disease to the pulmonary arterial tree alone is a very rare condition.     

Takayasu arteritis presenting as a pulmonary-renal syndrome

Takayasu arteritis is an uncommon disease with a variety of presentations. We report a case of Takayasu arteritis with a presentation of a pulmonary-renal syndrome in a 22-year-old woman. She presented in acute respiratory failure with hemoptysis and acute renal failure; interestingly, however, the renal biopsy was normal. Magnetic resonance angiography (MRA) showed significant narrowing in the distal abdominal aorta with bilateral renal and common iliac artery occlusions. Thoracic and abdominal angiogram confirmed MRA findings of type IV Takayasu arteritis. Percutaneous transluminal angioplasty of the left renal artery normalized kidney function. The initial presentation of Takayasu arteritis as a pulmonary-renal syndrome with severe acute renal failure and diffuse pulmonary hemorrhage is unusual; to our knowledge, this has not been described previously in the literature. We provide a clinical review of Takayasu arteritis and a discussion of systemic manifestations pertinent to the case.     

Pathological features of the pulmonary artery in Takayasu arteritis

Little attention has been paid to the pathological features of the pulmonary artery in Takayasu arteritis. Autopsy specimens of 6 cases of this disease were studied. Lesions were found in the aortic arch and its brachiocephalic branches in all cases and in both the aortic arch and thoracoabdominal aorta in 5 cases. The pathohistologic characters of the pulmonary artery were very similar to those of the systemic artery. Stenosis-recanalization, so-called blood vessels-in-blood vessels, of the pulmonary elastic arteries were found in four cases. These lesions were not observed in the systemic arteries, and most of the newly formed channels in them seemed to be branches of bronchial arteries. Luminal obstruction of pulmonary muscular arteries was observed in 4 cases, cellular arteritis of muscular arteries in 2 cases, and angiomatoid dilatation of small blood vessels in 2 cases. Thus in this study we found peculiar stenosis-recanalization lesions of the pulmonary elastic arteries, and also showed that the pulmonary elastic and muscular arteries are frequently involved in Takayasu arteritis. These findings suggest that pulmonary hypertension could influence morbidity and long-term mortality in this disease.     

Pulmonary hypertension in Takayasu arteritis

Takayasu arteritis (TA) is a rare chronic vasculitis primarily involving the aorta and its main branches. The disease affects women much more frequently than men, the ratio being 8:1. The onset occurs in the teenage years, always before age of 40. Aortic regurgitation is rare. The pulmonary artery stenosis may also rarely occur. We report the case of a 49-year old female patient with Takayasu arteritis who presented with severe left pulmonary trunk stenosis resulting in pulmonary hypertension, severe aortic regurgitation due to the dilatation of the ascending aorta, mitral insufficiency, critical left renal artery stenosis, and probably with left main coronary artery stenosis. No severe stenosis or occlusion in the mid portion of mid subclavian artery lesion were present. Because the patient presented with serious congestive heart failure, she underwent mitral valvuloplasty and aortic valve replacement. However, the patient died in early postoperative period due to pulmonary hypertension which failed to respond to pharmacotherapy.     

继发性肺动脉狭窄的临床诊治进展

继发性肺动脉狭窄的病因包括纤维性纵隔炎、结节病、大动脉炎和白塞病等,是导致第五大类肺动脉高压的主要病因.胸部CT肺动脉造影和肺血管三维重建不仅可以直观地描述肺动脉狭窄的部位、程度和范围,还有助于明确肺动脉狭窄的病因诊断.继发性肺动脉狭窄的治疗包括原发病的治疗和近端肺动脉狭窄的介入治疗.肺动脉狭窄区域肺灌注显著减低,同时合并中、重度肺动脉高压或右心功能不全是介入治疗的适应证.本文就继发性肺动脉狭窄的临床诊疗进展做了全面系统的阐述,对呼吸科肺血管病的临床实践具有重要的指导意义.     

Takayasu arteritis of subclavian artery in a Caucasian

Takayasu arteritis, an inflammatory and obliterative disease of medium and large arteries, is classified as a giant cell arteritis. It has a predilection for the aortic arch, its main branches, and coronary and pulmonary arteries. The early symptoms of Takayasu arteritis may be mainly systemic and may resemble polymyalgia rheumatica. The etiology is unknown but autoimmunity has been suggested to play a role. Diagnosis is based on symptoms, physical findings, and imaging, because tissue diagnosis is rarely feasible. Unlike atherosclerotic vascular disease, Takayasu arteritis affects primarily, but not exclusively, young women. Contrary to earlier reports, it is not limited to the women of Japanese origin but is present worldwide. The current report is of a Caucasian woman who presented with nonspecific complaints of upper back pain, weakness, malaise, and fatigue. Her physical examination revealed absent left radial pulse and a blood pressure differential, later confirmed by radiological imaging studies to be due to left subclavian artery stenosis consistent with Takayasu arteritis. The presentation and management of the patient is described, and Takayasu arteritis is succinctly reviewed.     

大动脉炎性肾动脉狭窄诊治进展

大动脉炎（TA）是一种少见的特发性大动脉慢性炎症性疾病,常可累及肾动脉导致肾动脉狭窄（RAS）、闭塞,进而引起严重高血压及心肾功能衰竭,致残致死率高。TA所致RAS的诊断和治疗理念尚存争议,因此,本文主要就TA性RAS的诊断和治疗做一综述。     

Patient with Takayasu arteritis presented as cardiogenic shock

Takayasu arteritis is a chronic inflammatory disease involving the aorta, its main branches and affects particularly young women. Symptomatic coronary artery disease and cardiogenic shock are rare signs of Takayasu arteritis. We describe a 47-year-old male patient in whom cardiogenic shock was the initial presentation of Takayasu arteritis with coronary, subclavian, celiac and total abdominal aortic occlusion.     

Pulmonary artery involvement in Takayasu arteritis: a retrospective study in Chinese population

Clinical Rheumatology - Takayasu’s arteritis (TA) may involve the pulmonary artery, which signifies a poor prognosis. This study investigated the features of TA patients with pulmonary artery...     

A successful surgical repair of pulmonary stenosis caused by isolated pulmonary Takayasu’s arteritis

A 48-year-old man underwent surgery to treat pulmonary stenoses of unknown origin. The right main pulmonary artery was severely stenotic, and the orifice of the left pulmonary artery was focally constricted. The patient was nearly asymptomatic, despite marked pulmonary hypertension and right heart strain. The pressure gradients beyond the stenotic regions were 88 mmHg bilaterally. The cause of the stenoses could not be established before operation. The right main pulmonary artery and ascending aorta were successfully replaced. It was suspected that the ascending aorta was involved because it was markedly thickened and adhered tightly to the right pulmonary artery. It was therefore also replaced. However, only the transected right pulmonary artery showed histological evidence consistent with a diagnosis of Takayasu’s arteritis. The postoperative course was uneventful. The patient was completely free of symptoms six months after the operation. To our knowledge, this is the thirteenth surgically treated case of isolated pulmonary Takayasu’s arteritis to be reported.     

Coronary artery lesions in Takayasu arteritis: Pathological considerations

This communication reviews the clinical and pathological features of coronary artery lesions in Takayasu arteritis. The incidence of coronary artery involvement has been reported to be 9% to 10%, and is observed mainly in autopsy cases because coronary artery disease is usually not evident until the occurrence of angina pectoris or myocardial infarction, or after the onset of congestive heart failure. On the basis of pathological features, the following three types of coronary artery lesions can be distinguished: type 1, stenosis or occlusion of the coronary ostia and the proximal segments of the coronary arteries; type 2, diffuse or focal coronary arteritis, which may extend diffusely to all epicardial branches or may involve focal segments, so-called skip lesions; and type 3, coronary aneurysm. Most of the coronary artery lesions in Takayasu arteritis are of type 1. Narrowing of the coronary arteries is mainly due to the extension of the inflammatory processes of proliferation of the intima and contraction of the fibrotic media and adventitia from the ascending aorta. In some cases, coronary stenosis may be caused by coronary arteritis as skip lesions in Takayasu arteritis, but even in these cases the lesions have been reported to affect mainly the proximal segments of the coronary arteries. Diffuse lesions of the coronary artery and coronary artery aneurysm seem to be very rare in Takayasu arteritis. Other causes of coronary ostial stenosis, coronary arteritis and coronary artery aneurysm are also discussed.     

Bilateral orbital pseudotumor in a patient with Takayasu arteritis: a case report and review of the literature

Inflammatory orbital pseudotumor is often associated with rheumatologic disorders. It has been reported commonly with ANCA-associated vasculitides, especially granulomatosis with polyangiitis (Wegener’s granulomatosis). There are also a few cases of large vessel vasculitis such as giant cell arteritis and Behcet’s disease. Here, we report a patient with undiagnosed Takayasu arteritis presenting with proptosis and diplopia, with later diagnosis of an inflammatory pseudotumor of the orbit. In this case, we believe extensive involvement of blood vessels, including bilateral pulmonary artery stenosis, and elevated inflammatory markers that show disease activity may be related to pseudotumor formation in Takayasu arteritis. Since this is an unusual and unreported presentation of the disease, better estimation of a causal relationship may be possible in the future with further information. In conclusion, although uncommon, this case highlights that orbital pseudotumor may be an important finding in Takayasu arteritis. For early diagnosis, better treatment, and good prognosis, it should be considered in patients presenting with ocular symptoms similar to the other vasculitides.     

Clinical features of main and peripheral pulmonary artery stenosis: significance of auscultation and phonocardiography]

Twenty-five patients with main and peripheral pulmonary artery stenosis were studied to determine the most frequently observed diagnostic sign of this abnormality regardless of the underlying etiologies, such as intrinsic or extrinsic narrowing of the pulmonary artery due to tumor, fibromuscular dysplasia, thromboembolism, angitis including the aortitis syndrome (Takayasu arteritis) and a variety of other disease entities. Among a variety of signs and symptoms, we emphasized the importance of cardiac auscultation and phonocardiography, which were often the initial diagnostic clues. The diagnostic features included a systolic murmur of pulmonary arterial origin and the behavior of the splitting of the second heart sound. The systolic murmur was often trans-systolic or continuous. In cases with left-sided cardiac murmurs (Takayasu arteritis, etc), the pulmonary systolic murmurs were not identified by auscultation alone and required phonocardiographic confirmation. In many cases the second heart sound was split and this was sometimes the first clue to the diagnosis. The split intervals varied, but were more marked in cases with pulmonary hypertension, and were accompanied by the accentuated pulmonic component (IIP). Phonocardiographic analysis disclosed that wide splitting was caused by the delayed appearance of IIP as well as the concomitant early appearance of the aortic component (IIA). It was concluded that, although the final etiological diagnosis is not identified, auscultation and phonocardiography provide important clues for further diagnostic and etiological studies of pulmonary artery stenosis.     

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.

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

Successful Early Immunosuppressive Therapy for Pulmonary Arterial Hypertension Due to Takayasu arteritis: Two Case Reports and a Review of Similar Case Reports in the English Literature

The efficacy of early immunosuppressive therapy without invasive therapy, such as endovascular or surgical revascularization, for pulmonary hypertension due to Takayasu arteritis (TAK-PH) remains to be elucidated. We herein report two cases of TAK-PH due to pulmonary arteritis successfully treated with early immunosuppressive therapy. A literature review of 42 cases of TAK-PH with pulmonary artery involvement showed that the cases treated with immunosuppressive therapy early after the onset (within 12 months) had a higher erythrocyte sedimentation rate and better outcome without invasive therapy than those treated later. TAK-PH may be successfully treated with immunosuppressive therapy without invasive therapy when diagnosed early with high disease activity.     

Takayasu arteritis in Korea: Clinical and angiographic features

Clinical and angiographic features of Takayasu arteritis were investigated in 129 Korean patients. This disease affects females more frequently than males, in a ratio of 6.6 to 1. Of the total number of patients, 51 were in the third decade, 27 in the fourth decade, and 23 in the second decade. Common clinical symptoms were headache (60%), exertional dyspnea (42%), dizziness (36%), and malaise or weakness (34%). Takayasu arteritis affected the abdominal aorta (46%) and descending thoracic aorta (37%) more frequently than the ascending aorta (1%) and aortic arch (2%) According to Ueno's classification based on aortographic findings, the 129 patients were divided into type I (37), type II (25), and type III (67). Among the 48 patients who had coronary angiography, 11 (23%) showed coronary arterial involvement. Because the clinical features are determined by the extent and severity of the specific artery involved in the occlusive phase of the disease, total aortography including coronary angiography is very important in the initial evaluation of Takayasu arteritis.     

Cerebrovascular manifestations of Takayasu arteritis in Europe

Objectives. Takayasu arteritis is well known as a cause of strokein Asia but has rarely been described in the Western world.Here we report the clinical and neuroimaging follow-up of aseries of patients with Takayasu arteritis from Europe. Methods. Seventeen consecutive patients who fulfilled the diagnosticcriteria for Takayasu arteritis of the American College of Rheumatologywere evaluated on follow-up by standardized neurological examination,sonography and MRI. Results. At follow-up almost 20 yr after onset of symptoms,the subclavian artery and the common carotid artery were oftenaffected. In addition, evidence of intracranial pathology wasfound in seven patients. In contrast to the severe vessel involvement,the neurological state was stable. Two patients had sufferedfrom stroke before the diagnosis was made and therapy was initiated,and one patient had recurrent transient ischaemic attacks. Intermittentdizziness was associated with pathology of the vertebral andbasilar arteries. However, clinical symptoms of subclavian stealsyndrome were rare. Conclusion. This case series shows that the clinical neurologicalcourse of Takayasu arteritis on treatment is benign in mostcases despite the severe vascular involvement. KEY WORDS: Takayasu arteritis, Vasculitis, Stroke, Cerebral ischaemia, Sonography, MRI * These authors contributed equally to this work.     

Pathological studies on Takayasu arteritis

Takayasu arteritis is a primary inflammatory disease of elastic arteries such as the aorta, its larger branches and the pulmonary artery trunk. According to our recent statistical survey of autopsy cases in Japan, the frequency of the disease in all autopsy cases was approximately 0.033% and the sex ratio was 1:4.5. The most frequent ages of the onset were 20–30 years, those of the death were 40–50 years. The latter was delayed about 20 years in comparison with a previous report. In the recent cases, the vascular lesions widely expanded. Luminal dilatation and aneurysm formation also increased in frequency, their ratio being approximately 57%. In the autopsy cases, the following active lesions were observed: (1) acute exudative inflammation (including suppuration), (2) chronic non-specific productive inflammation and (3) various types of granulomatous inflammation. These findings suggest that many triggers may play a role in the morphogenesis of Takayasu arteritis. The inflammatory lesions are produced in the media and adventitia through the vasa vasorum, and terminate in a diffuse or nodular fibrosis. New active lesions are often observed near the old fibrotic ones. This suggests that Takayasu arteritis may be a progressive disease. Intimal thickening of the peripheral branches from the affected arteries is very often observed. In consequence, secondary ischemic lesions are formed in various organs, especially the heart, brain and kidneys.