Fulminant development of mega-aorta due to Takayasu's arteritis: case report and review of the literature

Takayasu's arteritis is a rare inflammatory arteriopathy characterized by segmental involvement of the aorta, its major branches, and, occasionally, the pulmonary arteries. Arterial inflammation generally results in occlusion, but Takayasu's arteritis occasionally presents as aneurysm formation. Takayasu's arteritis generally afflicts young women and is most often characterized by an acute episode of systemic illness and neurologic symptoms secondary to stenoses of the carotid and vertebral circulation. We report an unusual case of Takayasu's arteritis in a 43-year-old man who presented with severe back pain and provide a brief review of the literature.     

Takayasu's arteritis

Takayasu's 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 and its branches and the pulmonary arteries. Unlike atherosclerotic vascular diseases, Takayasu's arteritis affects young women primarily. Early symptoms may be mainly systemic and may resemble polymyalgia rheumatica. The etiology is unknown, but infectious agents and autoimmunity have been suggested to play a role. Diagnosis is based on symptoms, physical findings, and angiographic findings, because tissue diagnosis rarely is feasible. The two cases reported here represent contrasting phases of Takayasu's arteritis.     

Pulmonary hypertension complicating systemic diseases other than connective tissue diseases

A COMPLICATION OF CERTAIN SYSTEMIC DISEASES: Pulmonary hypertension (PH) can complicate the progression of certain systemic diseases such as sarcoidosis, histiocytosis X and some vasculites. The mechanisms at the origin of PH are varied and always require rigorous analysis in order to optimise treatment. DEPENDING ON THE DISEASE: PH associated with sarcoidosis is essentially related to specific lung parenchymal fibrosis and is poorly responder to corticosteroids. Other mechanisms may be more rarely incriminated (compressive andenopathies, mediastinal fibrosis, florid sarcoidosis concomitant to a pulmonary occlusive vascular disease...). During histiocytosis X, the ventilatory limitation of these patients does not always correlate with the severity of the respiratory failure, suggesting the existence of a pulmonary vascular disease progressing independently of the pulmonary parenchymal lesions. The pulmonary artery damage during Takayasu's arteritis and other auto-immune pulmonary arteritis may lead to potentially life-threatening complications, notably through stenosis and/or obstruction of the pulmonary arteries. Pulmonary hypertension is exceptional during Wegener's disease or periateritis nodosa. CONCLUSION: PH can complicate the progression of certain systemic diseases. The physiopathological mechanisms responsible are unclear (specific parenchymal fibrosis, isolated vascular involvement...). Globally, available treatments are disappointing.     

Temporal arteritis with pauci-immune glomerulonephritis: a systemic disease

Temporal arteritis is easily diagnosed and responds gratifyingly to treatment. Renal complications are unusual, but nevertheless occur. Earlier, an association between pauci-immune glomerulonephritis and temporal arteritis was shown. We present a patient who clearly had temporal arteritis but also developed cerebral hemorrhage, pulmonary infiltrates related to granulomatous pulmonary vasculitis, and pauci-immune glomerulonephritis. We suggest that temporal arteritis is neither always localized nor temporal. Instead, the condition can be a lethal, systemic disease. Renal involvement in patients with temporal arteritis is not common and the presence of glomerulonephritis is rare [Jennette and Falk 1994]. Lenz et al. [1998] described a patient who developed vision loss, optic nerve atrophy, elevated erythrocyte sedimentation rate, a positive rheumatoid factor and terminal glomerulonephritis. The renal biopsy showed focal and segmental necrotizing glomerulonephritis, despite negative antineutrophil cytoplasmatic antibodies (ANCA), antinuclear antibodies and antiglomerular basement membrane antibodies. Giant cells were identified in the necrotic vessel walls within the kidney. Immunofluorescence was negative and a diagnosis of ANCA-negative pauci-immune glomerulonephritis was made. The patient did not respond to immunosuppression and developed end-stage renal disease. Although the clinical attributes were consistent with temporal arteritis, no temporal artery biopsy was done in that patient. We recently treated a patient with temporal arteritis and pauci-immune glomerulonephritis. Our patient's course was somewhat different in comparison to the patient described by Lenz et al. [1998].     

Pulmonary thromboendarterectomy in a patient with giant cell arteritis

This report describes the case of a young woman presenting with signs and symptoms of chronic thromboembolic pulmonary hypertension who underwent pulmonary thromboendarterectomy (PTE) with concomitant coronary artery bypass. She died in the intensive care unit 1 day postoperatively. At autopsy the patient was found to have giant cell arteritis of the pulmonary arteries and ascending aorta. It is important to differentiate this disease from chronic thromboembolic pulmonary hypertension because its management and that of systemic vasculitis differs considerably.     

A case of pulmonary artery bypass surgery for a patient with isolated Takayasu pulmonary arteritis and a review of the literature.

A 45-year-old female was presented with progressive dyspnea and bilateral leg edema. Pulmonary angiography revealed total occlusion of the right pulmonary artery and significant stenosis of the left pulmonary artery. The inferior lobar artery as well as the segmental arteries were well patent. No pathology was detected elsewhere at the aorta and its branches. The diagnosis of chronic pulmonary arterial occlusion by isolated Takayasu arteritis was made because of the characteristic pattern of angiographic findings and the presence of unusual shunt formation from the coronary artery to the peripheral portion of the pulmonary artery, as well as a characteristic presentation of HLA typing in blood analysis, which strongly suggested the diagnosis of Takayasu arteritis. To restore the pulmonary blood flow, we employed reconstructive surgery by means of bypass procedure, using PTFE graft. Postoperatively there was marked improvement in cardiopulmonary function and the quality of life of the patient. The graft was proved to be patent at long-term follow-up study. An extremely rare case of chronic occlusive pulmonary arteritis, which was surgically treated by means of bypass procedure, is reported herein, and a brief review of previous reports on this subject was attempted.     

Pulmonary artery replacement for pulmonary Takayasu’s arteritis

Takayasu’s arteritis is a chronic inflammatory disease that affects the pulmonary artery, as well as the aorta and its major branches. A 59-year-old man presented with a 2-month history of progressive exertional dyspnea. Further examination revealed marked wall thickening of the pulmonary trunk and bilateral proximal pulmonary artery, resulting in severe stenoses with high pressure gradient of 60 mmHg. The patient underwent graft replacement of the bilateral pulmonary artery and the pulmonary trunk with a 16-mm ring-supported extended polytetrofluoroethylene graft under extracorporeal circulation. Histopathological findings were consistent with Takayasu’s arteritis with pulmonary artery involvement. After the operation, the pressure gradient decreased to 6 mmHg and the patient was free from exertional dyspnea.     

An unusual clinical manifestation of Takayasu's arteritis: Spinal cord compression

Takayasu's arteritis is a chronic inflammatory vasculitis, involving mainly the aorta and its main branches and the pulmonary arteries, with characteristic of stenotic and occasionally dilated lesions. Neurologic manifestations of Takayasu's arteritis range from simple headache to catastrophic neurologic impairments, including visual loss, stroke and transient ischemic attack. However, spinal cord compression has never been described as a complication of Takayasu's arteritis. We describe a case of Takayasu's arteritis complicated by spinal cord compression due to thoracolumbar inflammatory epiduritis.     

Temporal arteritis. Clinical implications for the vascular surgeon.

Temporal arteritis is a systemic disease with local temporal artery symptoms, generalized constitutional symptoms and ocular involvement which affects the elderly. A study was undertaken to assess the clinical features of patients with temporal arteritis in a large multispecialty clinic practice. The study group consisted of 516 patients with clinical suspicion of temporal arteritis, of which 97 (18.8%) had a positive biopsy for arteritis. The records of these 74 females and 23 males were retrospectively reviewed for clinical implications of the disease. The average age of the cohort was 71.7 years, and male to female ratio was 1:3.2. There were 95 caucasians and 2 blacks. The most common clinical findings at presentation were abnormal temporal artery (65.9%), headache (64.8%), myalgias or arthralgias (46.6%), visual symptoms (37.1%) and fever (35.1%). Multiple symptoms were present in 97% of the patients. The erythrocyte sedimentation rate was > 50 mm per hour in 91% of patients. Corticosteroids were used to treat 95/97 patients. Twenty-seven (28%) of the patients completed treatment over an average 36.3 months. Sixty-eight (72%) other patients were either lost to follow-up, died, or continue on therapy. Complications of corticosteroid treatment occurred in 43 (44.3%) of patients, and complications of temporal arteritis occurred in 14 (14.4%). A review of biopsy data showed no difference in length of biopsy or yield of biopsy in the patients with positive and the patients with negative histology. Temporal arteritis is a systemic disease which responds well to corticosteroid treatment. Complications of the disease as well as of treatment make definitive diagnosis imperative.     

Two cases of Takayasu's arteritis occurring under anti-TNF therapy

Takayasu's arteritis is a granulomatous, large vessel vasculitis that affects the aorta, its major branches and the pulmonary arteries. Compelling evidence exists to support the notion that Takayasu's arteritis is a T-cell mediated process and that tumor necrosis factor alpha (TNFa) is an important factor in the pathogenesis of this disease. Moreover, encouraging results from recent studies support the use of anti-TNFa therapy for relapsing or resistant cases of Takayasu's arteritis. Here, however, we describe the case of two patients: one with seropositive rheumatoid arthritis, the other with HLA-B27 negative spondylarthropathy, who developed Takayasu's arteritis during treatment with TNFa inhibitors (adalimumab and golimumab respectively). This is the first report of Takayasu's arteritis in rheumatic patients under TNFa blocking agents which suggests the presence of different pathogenetic mechanism in a subgroup of patients with Takayasu's arteritis, as well as a potential role of TNFa blockers as triggers of this disease in some cases.     

Imaging diagnosis and interventional radiology in systemic vasculitis with particular emphasis on Takayasu arteritis: Part 1

This is a review article describing some new and interesting aspects in the diagnosis and treatment in systemic vaseulitis and demonstrating several cases that we have encountered. Particular emphasis will be put on Takayasu arteritis(also known as aortitis syndrome) most commonly observed in Japan and other oriental countries. CT and MRI better demonstrate the vessel wall abnormality of Takayasu arteritis than angiography. Importance of plain chest radiograph should also be kept in mind;it could provide a clue in the diagnosis of Takayasu arteritis in its early stage.     

Superficial temporal artery-middle cerebral artery anastomosis in a patient of temporal arteritis with internal carotid artery occlusion: a case report

Temporal arteritis is a rare systemic autoimmune disease and the arteritic process in this case of temporal arteritis involved large and medium-size arteries. Temporal arteritis with internal carotid artery (ICA) occlusion is very rare. We report a case of temporal arteritis with ICA occlusion following superficial temporal artery (STA) -middle cerebral artery (MCA) anastomosis, together with steroid therapy. A 73-year-old female presented with a headache, visual disturbance of left side, and suppression of activity. Left STA was inflammatory and overswelling. Magnetic resonance angiography (MRA) and angiography revealed occlusion of the left internal carotid artery (ICA) at the cervical portion and lowering of vascular reserve on PAO SPECT. Diagnosis as temporal arteritis was conclusive due to the clinical presentation, laboratory studies, and left temporal artery biopsy, so steroid pulse therapy was initiated. Inflammation of left STA disappeared after steroid therapy, but left ICA occlusion on angiography and lowering of vascular reserve on SPECT remained for 3 months afterwards. Because of this, STA-MCA anastomosis was performed. There were no complications after the operation and the donor artery has been patent for two years. Temporal arteritis with ICA occlusion that requires extracranial-intracranial bypass (EC-IC bypass) is very rare. STA-MCA anastomosis with steroid therapy is effective for the prevention of cerebral infarction.     

A Rare Cause of Renovascular Hypertension: Takayasu Arteritis with Only Renal Artery Involvement

Takayasu arteritis is a chronic inflammatory disease that affects mainly the aorta, main branches of aorta, and pulmonary arteries with unknown etiology. Disease affecting solely the renal arteries is rare. We will present a case that had hypertension, hypokalemia, and metabolic alkalosis where the etiology was type 2 Takayasu arteritis, affecting renal arteries.     

Ischemic Cardiomyopathy Due to Localized Takayasu Arteritis Treated by Heart Transplantation Following Left Ventricular Assisted Device Implantation: A Case Report

A 19-year-old Asian woman presented to the emergency department with ventricular fibrillation. Emergent coronary angiography revealed a 99% ostial stenosis of the left main coronary trunk, and percutaneous coronary intervention was performed. Takayasu arteritis was suspected, but fluorodeoxyglucose positron emission tomography scanning showed no active inflammation. Cardiac function was affected by ischemic cardiomyopathy, and an extracorporeal left ventricular assisted device was implanted under INTERMACS profile 1 status. Histopathology of the ascending aortic wall at the outflow anastomosis site showed no significant sign of Takayasu arteritis. The absence of systemic inflammation led to the replacement of the extracorporeal left ventricular assisted device with a Jervik 2000 as a bridge to transplant. An orthotropic heart transplant took place after a 39-month wait. Histopathology of the explanted heart revealed intimal and adventitial thickening with destruction of the elastic lamina localized at the sinus of Valsalva. Our final pathologic diagnosis was localized Takayasu arteritis. To counter the increased risk of stenosis or pseudoaneurysm formation at the vascular anastomosis site, anti-inflammatory therapy was essential in Takayasu arteritis. The post-heart transplant immunosuppression regime was considered stronger than that for Takayasu arteritis, and we therefore administered prednisolone, mycophenolate mofetil, and tacrolimus as standard protocol. There have been no signs of either relapse or rejection of transplantation for over 1 year. Further closed observation is required to clarify the long-term outcome of this rare condition with regard to heart transplantation.     

A girl with Takayasu arteritis associated with possible systemic lupus erythematosus

Takayasu arteritis is an inflammatory disease that affects the aorta and its main branches. Its etiology is obscure. Its association with systemic lupus erythematosus has been reported in the English literature in about 20 cases worldwide, and a relationship with a positive tuberculin test, either with or without tuberculosis, has also been mentioned. We report a pediatric patient who presented with renovascular hypertension secondary to Takayasu arteritis associated with a strongly positive tuberculin test and who subsequently developed possible systemic lupus erythematosus 8 months later.     

Report of a case with surprising etiology of renovascular hypertension

Takayasu arteritis is a chronic, idiopathic, inflammatory disease that primarily affects large vessels, such as the aorta and its main branches. Epidemiologically, it is found mostly in female patients and is more prevalent in Asian and Latin American countries. Disease may be heterogeneous in presentation. In this report, we present a different clinical expression of Takayasu arteritis in a young man who had hypertension as the sole manifestation of multiple critical arterial involvement with elevated inflammation markers but no other symptoms. A 28-year-old man was admitted with hypertension. There was no evidence for systemic vasculitis by history, serologic studies or other laboratory data. The acute-phase reactants were elevated with an erythrocyte sedimentation rate of 55 mm/h, and a C-reactive protein value of 22 mg/dl. Digital subtraction angiography showed multiple severe stenoses or occlusions of the branches of the abdominal aorta and arcus aortae together with bilateral renal artery involvement. The etiology of renovascular hypertension was found to be Takayasu arteritis with the presence of at least three criteria, as outlined by the American College of Rheumatology in 1990. Patients with Takayasu arteritis may have atypical clinical expression of the disease, and a diagnosis of Takayasu arteritis should be kept in mind in the differential diagnosis of renovascular hypertension in young subjects, even if they do not have associated symptoms of multiple arterial involvement.     

Aortic valve replacement for Takayasu's arteritis.

We report 12 cases of aortic valve replacement performed for Takayasu's arteritis and discuss the genesis of aortic regurgitation and the clinical outcome after aortic valve replacement. This group of twelve patients who underwent aortic valve replacement between April 1982 and March 1990 included four male and eight female patients, aged 24 to 67 years (mean age 48 years). Preoperative angiography showed systemic multiple stenoocclusive or aneurysmal dilated vascular lesions in addition to aortic regurgitation. The multiple lesions included a lesion in the aortic arch branch in nine (75%), in the pulmonary artery in seven (58%), an aneurysmal dilation in the ascending aorta of more than 6 cm in four (33%), a coronary lesion in four (33%), a thoracic aortic lesion in six (50%), and a lesion in the abdominal aorta and its visceral branch in six (50%). Simple aortic valve replacement alone was performed in two patients and in combination with another operation in ten patients, with aortic root reconstruction in two, ascending aortic plication in three, coronary artery bypass grafting in two, aortic arch branch bypass grafting in one, aortic arch branch bypass grafting and coronary ostium endarterectomy in one, and mitral valve replacement and ascending aortic plication in one. There was no operative death, and only one patient died later, 18 months after the operation, because of secondary amyloidosis. The postoperative recovery of the clinical status and cardiac function was good. Intraoperative observations suggested that aortic valve regurgitation may be caused by an extension of aortitis, although histopathologic examinations of the valve showed nonspecific findings. One of the characteristic problems in Takayasu's arteritis is the necessity for prednisolone administration in some patients preoperatively or postoperatively, or both. We conclude that aortic valve replacement for patients with Takayasu's arteritis is an effective and safe treatment. Our data related to the genesis of aortic regurgitation in Takayasu's arteritis remain insufficient to draw conclusions, and further analysis is planned.     

Type IV thoracoabdominal aortic aneurysm with lymphoplasmacytic aortitis and cystic medial degeneration in a 32-year-old patient with Marfan syndrome

Aortitis identified in approximately 12% of all thoracoabdominal aneurysms. The most common subtype of inflammatory aortitis is giant cell aortitis, followed by lymphoplasmacytic aortitis. Inflammatory aortitis may occur in isolation or as part of a systemic inflammatory disorder such as Takayasu arteritis, systemic lupus erythematosus, rheumatoid arthritis, and giant cell arteritis. Aortitis has not been described in patients with Marfan syndrome. We report the case of a 32-year-old man with Marfan syndrome and a strong family history of aneurysmal disease who presented with an asymptomatic Crawford type IV thoracoabdominal aneurysm. His aneurysm had no associated dissection, and surgical pathology revealed severe medial degeneration and lymphoplasmacytic aortitis. To our knowledge, this is the first report of such a finding in a patient with Marfan syndrome.     

Temporal arteritis: report of a case.

Temporal arteritis is a rheumatic disease that affects large and medium-sized arteries. It is a severe arteritis involving both the intima and media of the vessel and is a cause of headache that is frequently diagnosed erroneously as "atypical migraine." The patients have a burning or throbbing type of pain. Ultimately, there is localized inflammation or cellulitis over the swollen, tortuous artery. Jaw claudication, eye pain, photophobia, diplopia, and even blindness may accompany the temporal symptoms. As many as 20% to 60% of inadequately treated or untreated patients will lose their vision. Blindness may or may not be preceded by visual symptoms and funduscopic changes. A variety of systemic symptoms are also often present, including nausea, vomiting, chills, dizziness, and loss of weight. Temporal arteritis is not a common diagnosis in maxillofacial practice. We are presenting a case of temporal arteritis diagnosed after a biopsy. The patient eventually lost the vision from one eye.