Takayasu's Disease Research Articles

Prise en charge de la maladie de Takayasu

Takayasu disease is a large-vessels vasculitis involving the aorta and its main branches. The main symptoms are constitutionals signs and those in relation to vascular damage. Acute phase reactants can be discordant with the disease activity and imaging tools are crucial to determine the affected arterial sites and the vascular complications. The active inflammatory nature of arterial lesions can be difficult to determine, and the value of functional imaging such as FDG-PET should be discussed. The treatment includes the immunosuppressive treatment in case of active disease, cardiovascular risk prevention and management of adverse events related to immunosuppressive drugs. The arterial revascularization should also be considered if necessary and regular monitoring is mandatory.

Nonspecific aortoarteritis with lesion of the left branch of the pulmonary artery (Takayasu's disease)

Nonspecific aortoarteritis with lesion of the left branch of the pulmonary artery (Takayasu's disease)

Acute Coronary Syndrome in a Young Lady: A Rare Presentation of Takayasu Disease

Acute Coronary Syndrome in a Young Lady: A Rare Presentation of Takayasu Disease

Isolated Aortic Aneurysm with Aortic Valve Regurgitation in Takayasu Disease

Takayasu arteritis is a chronic inflammatory disease of the aorta, its main branches, and the pulmonary arteries. It is characterized by arterial stenoses and ischemic manifestations. Aneurismal dilatation of the ascending thoracic aorta associated with aortic regurgitation is rare in Takayasu disease. We report the cases of a 43-year-old woman with a Takayasu disease since 18 years, who presented a dyspnea and chest pain. Computed tmography and trans-thoracic echocardiography confirmed an ascending aortic aneurysm associated to a severe aortic insufficiency. The patient will underwent surgical replacement of the ascending aorta and the aortic valve.

189 - Repeated stenosis of both main coronary ostia in Takayasu disease treated by successive angioplasties

189 - Repeated stenosis of both main coronary ostia in Takayasu disease treated by successive angioplasties

Accelerated Atherosclerosis in Takayasu Disease: Case-Control Study

Aim: The aim of our study was to evaluate the atherosclerosis prevalence in Takayasu’s disease. Patients and Method: We analyzed prospectively in a case-control study a group of 64 patients with Takayasu disease aged 41 years [±11.94], a group of 50 rhumatoide arthritis (RA) patients. All women aged 45 years [±10.27] and a control group with an average age of 44 years [±12.63]. We performed a measurement of the intima-media thickness (IMT) in carotid level and we looked for the presence of carotid, aortic and femoral atheroma. Results: We found more plaques of atheroma in the Takayasu group; the carotid intima-media thickness was significantly higher in the Takayasu group and the RA group compared with the control group. The mean IMT of the Takayasu group was 0.91 mm [±0.368]. It was 0.76 mm [±0.151] for the RA group. And 0.71 mm [±0.141] for the controls. (P: 0.000). CRP > 12 mg was identified as the most strongly associated with the development of accelerated atherosclerosis in Takayasu’s disease and RA (p: 0.002) with an odds ratio of 14.5 (IC: 95%). Conclusion: The high prevalence of atherosclerosis discovered in Takayasu’s disease is not explained by the traditional vascular risk factors. It is not also explained by the corticoids and immuno-suppression treatments. The systemic inflammation associated with parietal local inflammation, observed in Takayasu arteritis appears to be responsible of premature and accelerated atherosclerosis.

Treatment of severe cerebral ischemia in takayasu's disease with arterial reconstruction

Objective To explore the effect of surgical bypass and the role of transcranial doppler (TCD) during the bypass operation. Methods A retrospective analysis was performed for the surgical data of cerebral ischemic Takayasu disease from January 2003 to December 2012 in single center. Forty one patients were divided into two groups: Group A with TCD during operation (n=22) and Group B without TCD (n=19). For group A, the main operation methods were 10 ascending aorta-biliteral axillary (brachial)/subclavian artery artificial vascular graft bypass-unilateral internal carotid autologous great saphenous vein grafting, 7 ascending aorta (aortic arch)-biliteral axillary (brachial)/subclavian artery artificial vascular bypass grafting, 3 ascending aorta-unilateral subclavian and unilateral carotid artery artificial graft bypass, and 2 ascending aorta-unilateral internal carotid artery autogenous saphenous vein grafting. For group B, the corresponding number was 8, 9, 1, and 1 case, respectively. On the basis of above-mentioned ascending aorta-biliteral axillary artery graft, there were 5 artificial graft-unilateral internal carotid saphenous vein-grafting in both groups. Results All operations were successful with a success rate of 100% and without death.All grafts remained patency when discharged, and all patients had improved cerebral ischemic symptoms. The effective rate of treatment was 100% in both groups, without statistical significance between two groups (P>0.05). Complications were reversible tongue for 5 cases in group A and 4 in group B (P>0.05), dizziness for 2 cases in group A and 9 in group B (P 0.05), eye swelling for 7 cases in each group (P>0.05), and cerebral hemorrhage for 0 cases in group A and 2 in group B (P>0.05). All patients were followed-up 3.5 to 13.5 years, mean (8.5±2.0)years. Symptom recurrence was 2 cases in group A and 1 in group B, and the recurrence rate of two groups had no difference (P>0.05). Conclusions Cervical arterial blood flow reconstruction is a safe and effective treatment for cerebral ischemia caused by Takayasu arteritis. TCD intraoperative monitoring can reduce postoperative complications caused by hyperperfusion significantly. Key words: Arteritis/SU; Carotid arteries/SU; Blood vessel prosthesis/AE; Brain ischemia/ET; Ultrasonography, doppler, transcranial

Carp properdin: Structural and functional diversity of two isotypes

Study was conducted to analyze children with systemic hypertension among admitted patients in age group between 3 and 18 years.Retrospective study over 5 years from January 2005 to January 2010.Department of Pediatrics NEIGRIHMS, a tertiary care referral hospital in North East India.Eighty-six children between 3 and 18 years admitted with hypertension. Children with transient hypertension due to intracranial causes were excluded.Out of 4445 total admissions, 86 children were admitted with hypertension (1.93%) of which 36 were males (41.86%) and 50 females (58.13%). Most number of cases presented between 11 and 15 years of age (50%). Most cases presented with blood pressure more than 99th percentile for age (87.69%). Renal cause of hypertension was seen in 59 cases followed by renovascular hypertension in 14 children. Acute glomerulonephritis was the most common of the renal causes (82.5%). Takayasu's disease accounted for most of the renovasular cases. Amlodipine was the most common drug used (23.4%). BMI was normal in 36 cases while in 11 cases it was more than 95th percentile.Hypertension is not a rare finding in the studied population. Most cases presented with severe hypertension. Renal and reno-vascular etiologies were the most common causes of hypertension.

Profile of hypertension in children: Experience from a tertiary care institute in North East India

Abstract Objectives Study was conducted to analyze children with systemic hypertension among admitted patients in age group between 3 and 18 years. Design Retrospective study over 5 years from January 2005 to January 2010. Setting Department of Pediatrics NEIGRIHMS, a tertiary care referral hospital in North East India. Participants Eighty-six children between 3 and 18 years admitted with hypertension. Children with transient hypertension due to intracranial causes were excluded. Results Out of 4445 total admissions, 86 children were admitted with hypertension (1.93%) of which 36 were males (41.86%) and 50 females (58.13%). Most number of cases presented between 11 and 15 years of age (50%). Most cases presented with blood pressure more than 99th percentile for age (87.69%). Renal cause of hypertension was seen in 59 cases followed by renovascular hypertension in 14 children. Acute glomerulonephritis was the most common of the renal causes (82.5%). Takayasu's disease accounted for most of the renovasular cases. Amlodipine was the most common drug used (23.4%). BMI was normal in 36 cases while in 11 cases it was more than 95th percentile. Conclusion Hypertension is not a rare finding in the studied population. Most cases presented with severe hypertension. Renal and reno-vascular etiologies were the most common causes of hypertension.

Dramatic Response to Tocilizumab Before Emergency Surgery in Severe Active Takayasu Disease.

A 19-year-old man was admitted in our tertiary care center in August 2014 for a 10-kg weight loss in a few months (48 kg, 1.80 m, and body mass index, 14.8 kg/m2), claudication in the lower limbs after a 500-m distance and a postprandial abdominal pain associated with mild renal insufficiency and proteinuria with no hypertension (100/80 mm Hg). The patient had no medical history and was an active smoker (tobacco and cannabis, estimated consumption: 2 pack-years). Laboratory parameters were elevated leukocyte count (13×109/L), C-reactive protein level (55 mg/L), creatinine (14 mg/L), and B-natriuretic peptide (4246 pg/mL). No thrombophilia, autoimmune disorders, or viral infections were identified. The patient underwent a B-mode Doppler ultrasound that showed multiple proximal arterial occlusions and stenoses of large vessels confirmed by a computed tomographic angiography: occlusions of bilateral subclavian arteries, superior and inferior mesenteric arteries, and right renal artery with kidney atrophy were found (Figure 1A). Furthermore, significant stenoses of celiac artery, left renal artery (Figure 1B), right internal iliac artery, and bilateral superficial femoral arteries were identified. A discrete circumferential thickening of the abdominal aorta was suggestive of aortitis. Cervical magnetic resonance angiography showed a complete occlusion of both subclavian and vertebral arteries (Figure 2). Transthoracic echocardiography revealed a severe left ventricular (LV) systolic dysfunction (LV ejection fraction …

Artériopathies périphériques juvéniles

Peripheral arterial disease (PAD) encompasses disease of all arteries of the body except the coronary arteries. The main etiology whatever the patient's age is atherosclerosis. Different etiologies can induce PAD especially when patients are younger than 50 years old and have no cardiovascular risk factors (smoking, hypertension, diabetes…). PAD that appears before 50 years old can be named juvenile PAD (JPAD) although there is no consensus about the definition. The aim of this work is to present the different etiologies of JPAD according to their hereditary, acquired or mixed origins. The following hereditary causes are addressed: Marfan syndrome, Ehlers-Danlos syndrome, homocystinuria, pseudoxanthoma elasticum, osteogenesis imperfecta “mid-aortic” syndrome. Among the acquired etiologies, inflammatory JPADs without extravascular signs such as atherosclerosis and Buerger's disease, inflammatory JPADs with extravascular signs as Takayasu's disease, Behçet's disease and Cogan's syndrome, JPADs like aortitis, embolic JPADs, iatrogenic JPADs, and mechanical or traumatic JPADs are described. Finally, mixed origins as thrombotic disease and fibromuscular dysplasia are presented. This work will assist clinicians in the diagnosis of JPAD.

Association sarcoïdose et maladie de Takayasu : à propos d’une observation

Vascular lesion of sarcoidosis is rare, it may precede, follow or occur with the symptoms of a Takayasu's disease. Many pathogenic mechanisms (infectious, genetic and immunological) participate in associating those two diseases. We describe the case of a 24-year-old female patient, hospitalized for a check up for mediastinal and cervical adenopathies, nasal obstruction with intermittent claudication in the upper limbs evolving over eight months the clinic examination shows an abolition of radial pulses. The final diagnosis was sarcoidosis with mediastinal and peripheral lymphadenopathy and a nasal lesion associated to a Takayasu arteritis (4 ACR criteria for Takayasu disease). We noticed a good clinical and radiological evolution after one year of oral corticotherapy. The association between sarcoidosis and Takayasu's disease remains rare, thus a differential diagnosis.

Inflammatory bowel diseases and Takayasu's arteritis: coincidence or association?

Takayasu's arteritis (TA) and inflammatory bowel disease (IBD) are rare diseases but there are case reports presenting their co-existence in the literature. The aim of this study was to investigate the relation between IBD and TA. We studied 52 consecutive TA patients (90.3% female); medical records of the patients were analyzed retrospectively and serum samples were taken during the control visits for anti-neutrophil cytoplasmic antibody (ANCA) and anti-saccharomyces antibody (ASCA) tests. Overall three (5.8%) of 52 patients had both IBD and TA. All were first diagnosed as IBD and the period between the diagnosis of IBD and TA was 9, 30 and 60 months, respectively. The age at diagnosis of TA was younger for the patients with IBD as compared to TA patients without IBD, but the difference was not statistically significant. Two patients had type-5 and one had type-2a TA. In 92 participants (52 with TA and 40 healthy controls) none had positive results for ANCA or ASCA. Anti-saccharomyces antibody and ANCA tests are not useful for predicting the association between TA and IBD. On the other hand, both diseases have similar patient characteristics and pathophysiology which make us suspect that there may be an interaction. If a patient with IBD under immunosuppressive treatment has ongoing symptoms such as fever, weight loss, hypertension or high acute phase reactants, TA may be the cause. Further trials are needed but their coexistence cannot be explained as incidental.

The lung in systemic vasculitis: radiological patterns and differential diagnosis.

The respiratory system may be involved in all systemic vasculitides, although with a variable frequency. The aim of our review is to describe radiographic and high-resolution CT (HRCT) findings of pulmonary vasculitides and to correlate radiological findings with pathological results. Lung disease is a common feature of antineutrophil cytoplasmic autoantibody-associated small-vessel vasculitides, including granulomatosis with polyangiitis (Wegener's), eosinophilic granulomatosis with polyangiitis (Churg-Strauss) and microscopic polyangiitis. Pulmonary involvement is less frequent in immune-complex-mediated small-vessel vasculitides, such as Behçet's disease and Goodpasture's syndrome. Pulmonary involvement associated to large-vessel (gigantocellular arteritis and Takayasu's disease) or medium-vessel (nodose polyarteritis and Kawasaki's disease) vasculitides is extremely rare. The present review describes the main clinical and radiological features of pulmonary vasculitides with major purpose to correlate HRCT findings (solitary or multiple nodules, cavitary lesions, micronodules with centrilobular or peribronchial distribution, airspace consolidations, "crazy paving", tracheobronchial involvement, interstitial disease) with pathological results paying particular attention to the description of acute life-threatening manifestations. A thorough medical history, careful clinical examination and the knowledge of radiological patterns are mandatory for a correct and early diagnosis.

Microscopic colitis in patients with Takayasu's arteritis: a potential association between the two disease entities.

The association of Takayasu's arteritis (TAK) and inflammatory bowel disease (IBD) has previously been reported in case series. Microscopic colitis (MC) has IBD-like symptoms with regard to clinical and histopathological feature. We aim to assess the presence of MC in TAK patients in this study. We cross-sectionally assessed TAK patients, between the ages of 18-65years, who were diagnosed according to the American College of Rheumatology (ACR) criteria. Disease activity was evaluated by Kerr's criteria. Age- and sex-matched irritable bowel syndrome (IBS) patients were selected as control group. All patients and controls have been interviewed for IBD and IBS symptoms using the questionnaires of WHO guideline and Rome III criteria, respectively. Lower endoscopic procedure was performed with at least five random biopsies taken from different colonic segments and the terminal ileum. A blinded expert pathologist evaluated the specimens for the features of MC. Thirty TAK patients (29 females and 1 male) with the mean age of 35 ± 11years (range, 20-59years) and 15 IBS controls with the mean age of 38 ± 13years were included in the study. TAK patients all fulfilled the MC criteria (three "complete" and six "incomplete" cases). MC was found to be significantly higher in active TAK patients in comparison to inactive group (67 vs 14%, p = 0.03, OR = 7.9). Our results show that there is an increased frequency of MC in TAK patients, and this is the first report on the association of TAK and MC.

Manifestations cardiaques de la maladie de Takayasu: à propos d’une observation et revue de la littérature

La maladie de Takayasu est une maladie vasculaire inflammatoire rare, touchant préférentiellement la jeune femme avec une atteinte prépondérante de l'aorte et ses premières branches de divisions. Nous rapportons le cas d'une atteinte ostiale du tronc commun gauche au décours d'un syndrome coronarien aigu ST négatif révélant une maladie de Takayasu et nous détaillerons les différentes manifestations cardiovasculaires de cette maladie.

Хвороба Такаясу (неспецифічний аортоартеріїт)

Резюме. В статье представлены современные представления об особенностях епидемиологии, етиопатогенеза, клиники, диагностики, лечения болезни Такаясу у детей и подростков. Болезнь Такаясу - это хроническое, рецидивирующее воспалительное заболевание аорты и ее основных веток неизвестного происхождения. Распространенность заболевания невысокая, чаще болезнь Такаясу встречается у женщин. Симптомы болезни Такаясу неспецифичны. В лечении болезни Такаясу используются глюкокортикостероиды, цитостатики, генно-инженерные биологические препараты, для устранения окклюзий сосудов используется хирургическое лечение. Ключевые слова: неспецифический аортоартериит, артериит Такаясу, дети, подростки, эпидемиология, этиология, патогенез, клиника, лечение.

Isolated abdominal aortic dissection in a young female patient

A 43-year-old woman presented with acute lower abdominal pain radiating to both sides of the groin. She had no history of smoking, drug abuse, surgeries, trauma, or any chronic diseases. There was no family history of vascular disease. On the admission physical examination, she was normotensive, had normal pulses on four limbs, and presented tenderness and pain on abdominal aortic palpation and in her lower abdomen. Contrastenhanced abdominal and thoracic computed tomography showed a distal infrarenal abdominal aortic dissection compromising both common iliac arteries (A) and a saccular dilation of the left common iliac dissected artery (B and C). The patient had partial remission of pain with opioids and elected to undergo an endovascular treatment. A coil embolization of the left internal iliac artery (the level at which the dissection ended) was performed, and then a unibody bifurcated endoluminal graft was deployed, covering most of the infrarenal aorta and both common iliac arteries. The patient recovered uneventfully, with total pain remission. The patient did not meet diagnostic criteria for Takayasu disease and had neither Marfan nor Ehlers-Danlos syndrome; her serologic inflammatory and infectious markers were negative, and she had no other radiologic signs of arterial disease of the aorta or its major branches. At 1-month follow-up, computed tomography angiography showed total thrombosis of the dissection’s false lumen as well as of the saccular dilation and patency of the infrarenal aorta and iliac arteries (D). Isolated abdominal aortic dissection, an extremely rare entity, is estimated to occur in 0.4% to 2% of all aortic dissections. It may be classified on the basis of etiology as iatrogenic, traumatic, or spontaneous. Most patients with spontaneous abdominal aortic dissections present with dissection of the infrarenal aortic segment, extending or not to the iliac arteries. Although there is no consensus in the literature on the treatment indications, most authors advocate treatment of all symptomatic patients and of those asymptomatic with radiologic evidence of aneurysmal dilation. There is no standardized technique, and although the endovascular approach used in this case was successfully undertaken, long-term follow-up is warranted to see how the device behaves in a young patient with small-caliber arteries.

Poumon et vascularites

Respiratory symptoms and specific radiological features are observed in many vasculitides. They are associated with extra-thoracic signs (nasal and sinus obstruction, skin disease, and acute renal failure). The main vasculitides not associated with ANCA include Behçet's disease with pseudo-aneurysm and pulmonary artery thrombosis and Takayasu disease involving large vessels with thickening of the wall of the aorta and the pulmonary artery. The hypocomplementemic McDuffie syndrome is rare and includes extensive urticaria, obstructive respiratory disease, and finally Goodpasture's syndrome with acute glomerulonephritis and intra-alveolar hemorrhage. The three ANCA-associated vasculitides are microscopic polyangiitis (MPA) with glomerulonephritis and intra-alveolar hemorrhage, granulomatosis with polyangitis (GPA) and pauci-immune glomerulonephritis, and eosinophilic granulomatosis with polyangeitis (EGPA) characterised by athma, hypereosinophilia, and systemic manifestations. Treatment of ANCA-associated vasculitides is based on high-dose corticosteroids, immunosuppressive therapy, and occasionally rituximab.

The Pathogenesis and Diagnosis of Thromboangiitis obliterans: Is It Still a Mystery?

Thromboangiitis obliterans (TAO) is a rare disease of unknown etiology that results in the occlusion of limb arteries located distally to the elbow and knee. Despite the fact that more than one hundred years have passed since its first description, knowledge on the pathogenesis of TAO and precipitating factors is still limited. Due to a lack of decisive noninvasive diagnostic methods and geographical differences in the prevalence of this condition, data on TAO epidemiology also remains sparse. This review presents important evidence on the pathogenesis and the course of the condition, as well as diagnostic modalities, with a focus on differential diagnosis. Theories on the pathogenesis of TAO include the theory of an infectious disease, coagulation disorders and injury to vascular endothelium resulting in activation of the inflammatory response. Differential diagnosis should exclude thoracic outlet syndrome, blue toe syndrome, infectious endocarditis, popliteal entrapment syndrome, Takayasu disease, primary and secondary systemic vasculitis, antiphospholipid syndrome, infection with the anaerobic Clostridium sp. bacilli and some less common conditions.