Atteinte rénale au cours de la maladie de Takayasu

Renal involvement in Takayasu's arteritis is frequent and worsens the progression of the disease. This is primarily a renal artery stenosis causing renovascular hypertension. The glomerular disease is exceptional. This study was undertaken to determine the clinical, radiological, biological features and therapeutic response in patients with kidney disease associated with Takayasu arteritis. A retrospective chart review was conducted on 11 patients (five men and six females), with a mean age of 31.1 years (19–40 years). The discovery of kidney disease preceded the diagnosis of Takayasu's arteritis in eight cases. Ten patients developed hypertension. Laboratory finding showed proteinuria in five cases of which one case was due to nephrotic syndrome. Renal failure was found in six cases including four cases in stage of terminal chronic renal failure. Impairment of the renal artery was present in nine patients, proximal in seven cases and distal in two cases, bilateral in five cases and unilateral in four cases. Narrowing renal artery was found in seven cases. The renal biopsy revealed membranoproliferative glomerulonephritis in one case and nephrosclerosis in another case. Eleven patients were followed for an average period of 155 months (3–335 months). Remission of nephrotic syndrome was concomitant with the remission of the disease. Seven patients developed outbreaks of Takayasu's arteritis of which six were in care. Relapse of nephrotic syndrome was concomitant with the outbreak of the disease followed by spontaneous remission of both diseases. Improved pressure was obtained in 5 cases and worsening renal function in seven cases. Death was observed in two cases.     

Treatment with Corticosteroid and/or Immunosuppressive Agents before Surgery can Effectively Improve the Surgical Outcome in Patients with Takayasu's Arteritis

Objective: To analyze the clinical outcome of corticosteroid and/or immunosuppressive treatment preoperatively in patients with Takayasu's arteritis. Patients and Methods: Forty-six patients with Takayasu's arteritis who received cardiovascular surgery between January 2010 and December 2015 in Beijing Anzhen Hospital were enrolled in this study. Their clinical characteristics, preoperative drug therapy, surgical treatment, and pathological examination results were retrospectively analyzed for the effect of drugs on outcome of the surgery. Results: All 8 patients with active disease prior to surgery had postoperative complications including one death due to stubborn perivalvular regurgitation induced heart failure during the perioperative period. Among 38 patients without active disease prior to surgery, only 4 patients (10.5%) had postoperative complications. Thirty-four patients showed symptomatic relief in the perioperative period, of whom 23 patients treated with corticosteroid and/or immunosuppressive agents preoperatively. Conclusion: The surgery can effectively improve the symptoms of patients with Takayasu's arteritis. Active disease of Takayasu's arteritis markedly increased risk for postoperative complication and resulted in poor outcome of the surgery. Treatment with corticosteroid and/or immunosuppressive agents before surgery can effectively control the patient's condition, improve the rate of remission, and effectively reduce the incidence of postoperative complications.     

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.     

Successful delayed bilateral renal revascularization during active phase of Takayasu's arteritis

Successful bilateral renal revascularization was performed 24 days after the development of angiotensin converting enzyme-inhibitor–induced bilateral renal artery thrombosis and anuric acute renal failure in a patient with Takayasu's arteritis. Excellent results were obtained after an unusually long ischemic time for a patient with active-phase disease. The outcome suggests that aggressive surgical revascularization can benefit patients with renal failure caused by renal arterial occlusion during the active phase of Takayasu's arteritis. (J Vasc Surg 1998;27:552-4.)     

18F-fluorodeoxyglucose-positron emission tomography scanning is a useful tool for therapy evaluation of arterial aneurysm in Behçet's disease

[¹⁸F]fluorodeoxyglucose-positron emission tomography (FDG-PET) has demonstrated its usefulness in giant cell arteritis, Takayasu's arteritis, and unclassified aortitis. FDG-PET imaging could be more effective than CT-scan or magnetic resonance imaging in detecting the earliest stages of vascular wall inflammation. Data are lacking regarding its accuracy for diagnosis and follow-up of vascular lesions in Behçet's disease. We report the case of a 32-year-old woman with history of Behçet's disease and fever of unknown origin revealing voluminous pulmonary arterial aneurysms. FDG-PET scan revealed a peripheral uptake of pulmonary aneurysms, underlying the inflammatory status of arterial aneurysms wall. Our patient was treated by glucocorticoids and intravenous cyclophosphamide and achieved remission of Behçet's disease. During follow-up, FDG-PET scan enabled to detect an early radiologic therapeutic response after 4 months, while CT angiography remained unchanged. We report the first evidence of usefulness of FDG-PET scan for therapeutic evaluation of arterial aneurysms in Behçet's disease. Further studies are warranted to determine the accuracy of FDG-PET scan in larger cohort of Behçet's disease patients with vascular involvement.     

A Case with Acute Renal Failure and Subsequent Nephrotic Syndrome

Nephrotic syndrome due to secondary amyloidosis is not so common, and the prognosis depends on primary disease. We report a case of secondary amyloidosis caused by Takayasu's arteritis. Sustained high fever and acute renal failure proceeded to the occurrence of nephrotic syndrome. Secondary amyloidosis was diagnosed by renal biopsy before the diagnosis of primary disease. She was completely recovered from nephrotic syndrome after two years' treatment with prednisolone, aspirin, and dimethyl sulfoxide. This rare case provides meaningful suggestions for the diagnosis and treatment of acute renal failure and nephrotic syndrome caused by secondary amyloidosis.     

Delayed dehiscence of modified mechanical Bentall 7 years postsurgery for Takayasu's arteritis

Following aortic surgery for vasculitis, the incidence and duration of onset of anastomotic breakdown is unclear. A case is presented of a young female patient with Takayasu's arteritis (TA) who was found to have frank dehiscence of a modified Bentall repair 7 years after surgery. The case highlights (i) the lack of normal healing following aortic surgery in TA, and (ii) need to differentiate imaging protocols for patients with defined vasculitis as opposed to degenerative aortic conditions. A recommendation is made for appropriate surveillance imaging modality alternating between computed tomography and magnetic resonance imaging in the often young patient population affected by vasculitis.     

Aortic connector for coronary revascularization in a patient with Takayasu's disease

Takayasu's disease affects the aorta and its major branches including coronary arteries, some of which may require coronary artery bypass grafting (CABG). However, calcification of the aorta affected by Takayasu's disease often makes proximal anastomosis of a vein graft very difficult. In addition, since the major branches of the aortic arch are also frequently affected by it, the internal mammary arteries are unsuitable for use in CABG. We report a 60-year-old woman with stenosis of the left main coronary artery and heavy aortic calcification caused by Takayasu's disease whose severe angina was successfully relieved by off-pump CABG using mechanical aortic connectors for proximal vein graft anastomoses.     

Aortic aneurysm and aortic regurgitation following aortic valve replacement due to Takayasu's arteritis

A 26-year-old man who underwent aortic valve replacement for aortic regurgitation due to Takayasu's arteritis 2 years earlier experienced left amaurosis persisting for some minutes. Computed tomography showed aneurysmal dilation of the ascending aorta to a diameter of 60 mm and occlusion of the left carotid artery. Cardiac echography showed perivalvular leakage. Following administration of a calcium antagonist, the patient's amaurosis subsided and brain bloodstream scintigraphy showed no abnormalities. We resected the aneurysm instead of using Bentall's operation. Following an uncomplicated postoperative course, the patient was discharged 21 days after surgery and echocardiography has shown no perivalvular leakage to date.     

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??Treatment of lower limb ischemia in Behcet disease and Takayasu’s arteritis CHEN Yue-xin, ZHOU Xiang, LIU Chang-wei. Department of Vascular Surgery, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing 100730, China
Corresponding author: LIU Chang-wei, E-mail??liucw@vip.sina.com
Abstract Although peripheral arterial atherosclerosis is known to be a major etiologic factor for lower limb ischemia, if it occurs in young patients in the absence of traditional risk factors for atherosclerosis, other non-atherosclerotic conditions must be considered, such as vasculitides, fibromuscular dysplasia, lower limb artery embolism or aortic dissection. Vasculitides refer to a heterogenous group of disorders that are characterized by inflammation within blood vessels and thus result in organ perfusion dysfunction and tissue necrosis. Vasculitides can involve multiple systems, its clinical manifestation can take on various looks, and diagnosis and treatment can usually be rather difficult. Lower limb ischemia could be part of clinical picture in giant-cell arteritis, Takayasu’s arteritis, Buerger's disease, polyarteritis nodosa or Behcet disease. Surgeons should pay attention to the treatment of Behçet disease and Takayasu’s arteritis, so as to improve the awareness of vasculitides as a rare cause of lower limb ischemia.     

Vasculitis working group: Selected unanswered questions related to giant cell arteritis and anti-neutrophil cytoplasmic antibody-associated vasculitis

Evidence to guide assessment and management of patients with vasculitis is lacking for many important clinical questions. The evidence surrounding several common questions about management of vasculitis was reviewed. Patients with giant cell arteritis (GCA) are at risk for developing extra-cranial large vessel inflammation. Clinicians should be aware of this complication and search for large vessel involvement in patients with GCA who have ischemic symptoms. Research is needed to define optimal strategies to identify patients with such complications. Because of the hazards of chronic corticosteroid use, alternative therapies for patients with GCA have been sought but thus far no clear alternatives have been identified. Anti-neutrophil cytoplasmic antibodies (ANCA) are associated with small-vessel vasculitis, including Wegener's granulomatosis and microscopic polyangiitis, but changes in ANCA titers should not be used as a surrogate biomarker for disease activity. Several immunosuppressive agents can be used for maintenance therapy after induction of remission in patients with ANCA-associated vasculitis, with no firm evidence that one agent is superior to others. Collectively, this review shows that more research is needed to provide a firmer body of evidence to support clinical decision-making for patients with vasculitis.     

Increased incidence of spondyloarthropathies in patients with Takayasu arteritis: a systematic clinical survey

ObjectivesTakayasu arteritis and Spondyloarthritis are two distinct inflammatory diseases that affect the same age periods. Increasing number of reports on co-incident Takayasu arteritis-spondyloarthritis cases in literature raised the hypotheses about their association. The purpose of this study is to evaluate the incidence of spondyloarthropathy spectrum diseases in Takayasu arteritis patients.MethodsDetailed clinical and demographic features of Takayasu arteritis patients were recorded and all were screened meticulously for the presence of spondyloarthropathy features following recommendations of Assessment of SpondyloArthritis international Society. Patients were questioned for inflammatory back pain, enthesitis, uveitis, inflammatory bowel disease, peripheral arthritis, and investigated accordingly with HLA-B27, plain X-rays and sacroiliac magnetic resonance imaging.ResultsA total of 69 Takayasu arteritis patients (65 female, 94.2%) were enrolled. After detailed investigation, 14 (20.3%) Takayasu arteritis patients fulfilled the Assessment of SpondyloArthritis international Society criteria for Spondyloarthropathy. Two of 14 (14.2%) spondyloarthropathy patients were positive for HLA-B27. Type 1 and type 2 Takayasu arteritis were more common in patients with diagnosis of both Takayasu arteritis and spondyloarthropathy than those without spondyloarthropathy. Most of patients with diagnosis of both these diseases required biologic therapies than patients with diagnosis of Takayasu arteritis alone (64.3% vs 29.1%, P = 0.014) due to refractory Takayasu arteritis.ConclusionOur results suggest a significant association between Takayasu arteritis and spondyloarthropathy. Possible shared genetic or immunopathogenic processes may explain this association, which merits further investigations.     

白塞病及大动脉炎下肢缺血的诊疗要点

动脉粥样硬化是下肢缺血最常见原因,但对于缺少心脑血管疾病危险因素的年轻病人,尚应考虑其他少见病因,如血管炎、纤维肌性发育不良、下肢动脉栓塞、主动脉夹层等。系统性血管炎以血管壁炎性反应为主要病理改变,从而导致相应组织器官的供血障碍和组织坏死。血管炎可累及全身各个系统,临床症状复杂,诊断与治疗棘手。下肢缺血可并发于巨细胞动脉炎、大动脉炎、Buerger病、结节性多动脉炎和白塞病等。对于少见病因如白塞病及大动脉炎并发的下肢缺血,值得引起临床医生的重视。     

Takayasu's arteritis with renovascular hypertension: results of surgical treatment.

Renal artery stenosis caused by Takayasu's arteritis is an important cause of hypertension in young patients in the Far East. The role of surgery in Takayasu's arteritis is not as well-defined as in atherosclerosis or fibromuscular dysplasia. In this retrospective review, the author reports the results of 19 renal artery reconstructions in 12 young patients (median age 23.5 years, range 10-46 years) presenting with renovascular hypertension and Takayasu's arteritis, and discusses the different surgical options. The procedures performed included aortorenal bypass using vein (five), aortorenal bypass using polytetrafluoroethylene (PTFE) (eight), iliorenal bypass using vein (four), reimplantation of renal artery (one) and aortic replacement graft-renal bypass (one). Postoperatively, all 12 patients had a successful outcome with improved hypertension. There was no perioperative mortality, and complications included two early graft thrombosis and one late graft occlusion. These results support the view that surgical treatment for renovascular hypertension in Takaysu's disease is safe and effective.     

Interleukin-1 blockade in refractory giant cell arteritis

Giant cell arteritis is a primary large-vessel vasculitis characterized by an arterial wall inflammation associated with intimal hyperplasia leading to arterial occlusion. Glucocorticoids remain the mainstay of giant cell arteritis treatment. However, relapses and glucocorticoid-related complications are frequent and therapeutic options for refractory giant cell arteritis are quite limited. Like tumor necrosis factor-α and interleukin-6, interleukin-1β is also highly expressed in inflamed arterial walls of patients with giant cell arteritis and may contribute in the pathogenesis of this disease. We report treatment of three cases of refractory giant cell arteritis successfully treated with anakinra, an interleukin-1 blockade therapy. Anakinra was effective for all patients, yielding improvement in their inflammation biomarkers and/or in their symptoms, as well as a disappearance of arterial inflammation in PET/CT for two of them.     

Mini-Laparotomy for Superior Mesenteric Artery Aneurysm Due to Takayasu's Arteritis

Superior mesenteric artery aneurysm (SMAA) is reported to be the third-most common type of visceral aneurysm (VA), accounting for 5% of all VAs. The etiology of SMAA is commonly thought to be infection, and it usually exists in the proximal part of the superior mesenteric artery, which is suitable for endovascular treatment. We herein report an extremely rare case of the distal part of SMAA caused by Takayasu''s arteritis (TA), which was successfully resected using a mini-laparotomy method without impairing the intestinal blood supply. A 51-year-old woman was admitted to our hospital with sustained fever and lower back pain. Physical examination showed that she had a discrepancies in blood pressure between both arms. Contrast-enhanced whole-body computed tomography showed stenosis of the thoracic aorta and an aneurysm located in the distal part of the superior mesenteric artery. The diameter of the aneurysm was 4.5 cm. The aneurysm was resected via 4-cm mini-laparotomy, and the vascularity of the intestine was successfully preserved. The postoperative course was uneventful, and the patient was diagnosed as having TA based on both clinical and pathologic findings. Additional corticosteroid therapy was started to treat the arteritis, and at 3-month follow-up she was without critical incidents. Mini-laparotomy is a safe and less-invasive approach to treat SMAA, especially when the lesion is located in the distal part of the artery.Key words: Superior mesenteric artery aneurysm, Takayasu''s arteritis, Mini-laparotomyVisceral aneurysms (VAs) are rare, with an incidence of 0.01% to 2% in routine autopsies.^1,2 However, the recent widespread use of computed tomography (CT) has led to frequent incidental detection of them.³ Superior mesenteric artery aneurysm (SMAA) is reported to be the third-most common type of VA,⁴ accounting for 5% of all VAs.⁵ Although SMAA shows few specific symptoms, several reports have revealed that it has a very high risk of rupture and mortality.^4,5 SMAA is commonly located in the proximal part of the SMA,^4,6 which is suitable for endovascular treatment (ET).^7,8 Here, we report a rare case of SMAA associated with Takayasu''s arteritis (TA), located in the distal part of the SMA and treated by surgical resection using the mini-laparotomy method.     

Effectiveness and safety of tocilizumab in patients with refractory or severe Takayasu's arteritis: A prospective cohort study in a Chinese population

ObjectiveTo assess the effectiveness and safety of tocilizumab (TCZ) in treating severe/refractory Takayasu's arteritis (TAK).MethodsA prospective cohort study was started on 1 November 2013 and terminated on 10 June 2020. Thirty-seven patients diagnosed as severe/refractory TAK, treated with TCZ combined with or without immunosuppressors were enrolled. Treatment response (complete remission (CR) and partial remission (PR)), imaging progression and side effects were analyzed at 6-month treatment. Disease flare was analyzed during the remaining follow-up.ResultsThe CR and RR rates were 70% and 88% at 6 months of TCZ treatment, respectively. Glucocorticoids was tapered from 30.0 (20.0–40.0) to 15.0 (10.0–15.0) mg/day at 6 months. Younger patients (≤ 26 years) (OR = 14.6, 95% CI 1.27–170.4, P < 0.05) and those with involvement of bilateral carotid arteries or vertebral arteries (OR = 14.6, 95% CI 1.27–169.1, P < 0.05) might show a better response to TCZ at 6 months. Combined therapy of immunosuppressors had no significant effects on the effectiveness of TCZ at 6 months. Among the total 23 patients with CR at 6 months, 14 cases discontinued TCZ therapy after 6 months, and disease flare was observed in six ones (43%), with medium flare at 7 (7–9.8) months. One patient (11%) who continued TCZ therapy suffered disease flare at 8 months. Infections were the most commonly observed side effects (38%), with four patients discontinuing TCZ treatment due to severe infections.ConclusionTCZ treatment achieved a favorable response with acceptable adverse effects for TAK.     

Non-traumatic cervicomediastinal vascular lesions. A clinicopathological study in the different populations of Natal

Apart from those suffering from vascular trauma, 334 patients with cervicomediastinal lesions have been treated at Durban Metropolitan Hospitals over a period of 8 years. Sixty-seven of these were black (20%), 79 Indian (24%), 10 coloured (3%) and 178 white (53%). Atherosclerotic disease was found in 50% of black, 80% of Indian and 99% of white patients; the remaining patients suffered from miscellaneous conditions, such as arteritis, fibromuscular dysplasia, mucoid degeneration and radiation. Aneurysmal disease was found in 33% of blacks, but only 2% of Indians and 0.6% of whites; the remainder had occlusive lesions. More blacks presented with complete stroke (16%) than in the other population groups, who presented most frequently with episodic neurological dysfunction. It is concluded that atherosclerosis is as common as arteritis in blacks, while it is the most common precipitating factor for cervicomediastinal lesions in the other two groups. Aneurysms and complete stroke are also common in blacks.     

Reno-vascular hypertension in childhood: a nationwide survey

Renovascular disease accounts for 8–10% of all cases of paediatric hypertension, whereas, in adults, its incidence is approximately 1%. The Turkish Paediatric Hypertension Group aimed to create the first registry database for childhood renovascular hypertension in Turkey. Twenty of the 28 paediatric nephrology centres in Turkey responded to the survey and reported 45 patients (27 girls, 18 boys) with renovascular hypertension between 1990 and 2005. The age at presentation ranged from 20 days to 17 years. The mean blood pressure at the diagnosis was 169/110 mmHg. Chief complaints of symptomatic patients were headache (38%), seizure (18%), epistaxis (4%), growth retardation (4%), cognitive dysfunction (4%), polyuria (2%), palpitation (2%), and hemiplegia (2%). Renovascular hypertension was found incidentally in 11 children. The diagnosis of renovascular hypertension was established with conventional angiography in 39 patients, MR angiography in three, CT angiography in two, and captopril diethylene triamine penta-acetic acid (DTPA) scintigraphy in one patient. Twenty-one children had bilateral renal artery stenosis and 24 had unilateral renal artery stenosis. Of these, 14 (31%) had fibromuscular dysplasia; 12 (27%) Takayasu’s arteritis; six (13%) neurofibromatosis; two (5%) Williams syndrome; one (2%) Kawasaki disease; one (2%) mid-aortic syndrome; one (2%) extrinsic compression to the renal artery, and eight (18%) unspecified bilateral renal artery stenosis. Hypertension was controlled with antihypertensive drugs in 17 patients. Percutaneous transluminal angioplasty (PTRA) or surgery had to be performed in 28 patients: PTRA in 16 patients, PTRA + surgery in one patient and surgery in 11 patients (four nephrectomies). The importance of vasculitic disease, especially Takayasu’s arteritis, should not be underestimated in children with renovascular hypertension.     

Arm ischemia secondary to giant cell arteritis

Six cases of arterial insufficiency of the arm secondary to giant cell arteritis are described, all in elderly white women. The clinical presentation of the occlusive disease ranged from an asymptomatic incidental physical finding to an alarming picture of severe ischemia. All patients were treated with steroids and had subsequent stabilization or improvement of extremity symptoms. Vascular reconstruction was also performed in two patients, one of whom developed rest pain after graft occlusion. Another patient had a cerebral infarction while taking prednisone, despite control of large vessel vasculitis. This study indicates that giant cell arteritis should be considered in cases of occlusive disease of the arms, especially in elderly women. Giant cell arteritis is a seriously morbid and potentially fatal disease which justifies a thorough evaluation when sufficient evidence is present to suggest the diagnosis. The response to steroids is usually adequate to eliminate the need for early surgical intervention.