A case of Takayasu arteritis complicated with glomerulonephropathy mimicking membranoproliferative glomerulonephritis

We report on a 65-year-old Japanese woman with Takayasu arteritis who developed severe proteinuria, hypertension, and renal dysfunction. Renal angiography demonstrated moderate irregular narrowing of both renal arteries. Renal biopsy showed glomerulonephropathy mimicking membranoproliferative glomerulonephritis (MPGN) with glomerular capillary wall thickening (double contour) accompanied by mesangial cell proliferation and moderate increase of mesangial matrix without deposits of C3. Electron microscopy showed no subendothelial deposit and no circumferential mesangial interposition (CMI), and these findings are different from MPGN. In this report we present a case of Takayasu arteritis associated with glomerulonephropathy mimicking MPGN.     

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.     

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.     

大动脉炎合并冠心病的临床特点和危险因素分析

目的 探讨大动脉炎(TA)合并冠心病(CAD)的临床特点并分析其危险因素.方法 回顾性分析2012年1月至2019年3月在北京安贞医院风湿免疫科住院的TA患者101例,分为TA合并CAD组28例和TA不合并CAD组73例.比较两组患者的一般资料、临床特点和预后情况,并采用logistic回归分析TA合并CAD的危险因素...     

Immunopathogenesis of Takayasu arteritis

Takayasu arteritis is a common cause of renovascular hypertension in India. Sensitization to infective agents, particularly mycobacterium tuberculosis or antoimmune disturbances have been incriminated in its pathogenesis. Twenty patients of Takayasu arteritis along with groups of normal individuals, patients of essential hypertension, autoimmune disorders, tuberculosis, and healthy tuberculin reactors were studied. Besides detailed immunological profiles including LE cell phenomenon, serum complement C3 levels, antibodies to single (SS) and double stranded (DS) DNA, rheumatoid factor, lymphocyte subsets, blast transformation to antigens including, phytohemagglutinine, PPD, pokeweed, and purified human aortal antigen (PHAA) were examined. Soluble protein from human aorta was fractionated into 9 peaks by DEASE-52 and Sephadex G-75 chromatography, and 25 µg of major protein fraction-II was used for blast transformation study. Blast transformation by PHAA was higher in Takayasu arteritis as compared to all other groups (P < 0.05).=" blast=" transformation=" to=" ppd=" showed=" wide=" variation=" in=" all=" the=" groups,=" and=" was=" significantly=" higher=" only=" in=" tuberculin=" reactors=" as=" compared=" to=" controls.=" these=" observations=" support=" aortal=" sensitization=" to=" phaa=" playing=" a=" role=" in=" pathogenesis=" of=" takayasu=" arteritis=" and=" do=" not=" relate=" tuberculosis=" to=" takayasu=" arteritis,=" at=" least=" immunologically.=" in=" addition,=" the=" ratio=" of=" cd-4=" positive=" to=" cd-8=" positive=" lymphocytes=" changing=" in=" favor=" of=" the=" former=" and=" the=" concomitant=" increase=" in=" b=" lymphocytes=" favor=" the=" presence=" of=" autoimmune=" disturbances=" in=" takayasu=">     

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.     

Clinical gynecologic features of pregnancy in Takayasu arteritis

Takayasu arteritis is a non-specific chronic inflammatory vascular disease of unknown etiology. Since the incidence of this disease in the child-bearing years is relatively high, the management of pregnancies with this disease is of great importance in clinical obstetrics. This study is aimed at discussing the maternal management and obstetrical outcome, based on the clinical data obtained from 23 pregnancies of 15 patients treated in our hospital in the past 12 years. Since the disease was in the active state, artificial abortions were conducted in four cases in the 1st trimester of pregnancy. Among the remaining 16 cases, 3, who exhibited neither hypertension nor other complications, vaginally delivered neonates weighing 2,660–3,100 g with Apgar scores of nine after 37 weeks' gestation. C-sections were performed for 13 patients who showed sustained hypertension or/and developed other vascular disorders. Their gestational periods ranged from 34 to 40 weeks and the body weight of the infants varied from 1,425 to 3,024g. No adverse influence of pregnancy and delivery on Takayasu arteritis was detected in the puerperium of any patients. It is suggested that the state of Takayasu arteritis in early pregnancy and the magnitude of blood pressure elevation in the late gestational period are the most critical and definitive factors in determining the management of pregnancy of a patient with Takayasu arteritis. Cooperative managements by the specialists in obstetrics, internal medicine, and perinatology are required to provide a satisfactory clinical outcome.     

Takayasu arteritis: burnt out or not?

We report a case of a 57‐year‐old woman with Takayasu arteritis. She had a long history of poorly controlled hypertension and now suffers from severe ischemic cardiomyopathy and lower limb claudication. She did not receive immunosuppressive treatment as there were no signs of inflammation clinically or biochemically. Inspite of the latter, her symptoms have progressed. This case highlights the importance of improved modes of detecting inflammation as well as the need for strict management of vascular risk factors to control atherosclerosis, which has an increased incidence in patients with Takayasu arteritis.     

Typical angina in a patient with Takayasu arteritis

Takayasu arteritis (TA) is a well‐reported form of large‐vessel vasculitis that primarily affects the aorta and its major branches. Cardiac manifestations of TA have been reported and can include typical angina secondary to coronary artery involvement; however, typical angina in the setting normal coronary arteries is uncommon. We describe a case of typical angina in a patient with TA with normal coronary arteries secondary to diastolic hypotension, in the absence of significant aortic regurgitation, likely from poor aortic distensibility and elevated left ventricular end‐diastolic pressure.     

SAPHO syndrome with Takayasu arteritis successfully treated with tofacitinib

SAPHO syndrome is an autoinflammatory disease with a variety of clinical manifestations, which may be accompanied by other systemic inflammatory diseases in addition to the typical manifestations of common synovitis, acne, pustulosis, hyperostosis, and osteitis. Here, we report the first case of SAPHO syndrome combined with Takayasu arteritis.     

Crohn’s disease and Takayasu’s arteritis: An uncommon association

Takayasu’s arteritis(TA)and Crohn’s disease(CD)are two rare autoimmune disorders;however some reports describe the presence of both diseases in the same patient.This finding has suggested the possibility that both diseases could share some common etiologic origin.We describe a case of a 13-year-old male affected by CD characterized by fever,diarrhea,weight loss,abdominal pain and elevation of inflammatory markers.Clinical and histological features from colonic specimens were consistent with CD.Treatment with steroids and azathioprine was started,however disease flared every time steroids were tapered.One year later,while still on treatment,he came back to our attention for dyspnea at rest and at night,tiredness and weakness.At physical examination a diastolic heart murmur was found as well as a left carotid artery bruit.A transthoracic echocardiography showed mild aortic valve insufficiency,left ventricular hypertrophy and a dilated ascending aorta with same findings at the aortic arch.A computed tomography scan showed abdominal aortathickening,dilated thoracic aorta and the presence of a thoracic aortic aneurysm.TA associated with CD was diagnosed and medical treatment with cyclophosphamide,steroids and aminosalicylic acid was started,with good clinical response at 6 mo follow-up.We discuss the presence of possible common causes for the two diseases and the importance of differential diagnosis in those patients characterized for intractable disease.     

Endoscopic scoring systems for inflammatory bowel disease: pros and cons

Endoscopy plays a pivotal role for diagnosis and assessment of disease activity and extent in patients with inflammatory bowel diseases. International guidelines recommend the use of endoscopic scoring systems for evaluation of the prognosis and efficacy of medical treatments. Ideal scoring systems are easy to use, reproducible, reliable, responsive to changes, and validated in different clinical settings in order to guide therapeutic strategies. However, currently available endoscopic scoring systems often appear as complex for routine endoscopy and suffer from insufficient interobserver agreement and lack of formal validation which often limit their use in clinical trials. Here, we describe the role of endoscopic scoring systems in inflammatory bowel diseases focusing on pros and cons in the era of advanced endoscopic imaging and mucosal healing.     

Takayasu arteritis developing during treatment of ulcerative colitis with infliximab

A 22-year-old female with ulcerative colitis that was successfully treated with infliximab (IFX), and remained stable following tapered discontinuation of prednisolone, developed anterior neck pain and elevation of C-reactive protein following her fourth administration of IFX. She was diagnosed with Takayasu arteritis (TA) based on neck ultrasound and computed tomography angiography. This is the first report describing the development of TA during treatment of UC with IFX.     

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.     

Medical treatment of Takayasu arteritis

The guidelines for medical treatment of Takayasu arteritis established in 1987 by the Systemic Vascular Disorders Research Committee, Ministry of Health and Welfare of Japan are presented. The first part of the guidelines concerns treatment with adreno-corticosteroids and the second part concerns other medical treatment. A review of the literature referring to steroid therapy and other medical treatment of Takayasu arteritis is also included.     

Takayasu arteritis with multiple cardiovascular complications

A 60-year-old Japanese woman first presented in 1990 with effort angina. She underwent coronary angiography and was diagnosed with bilateral coronary ostial stenosis and Takayasu arteritis. Coronary artery bypass graft surgery (CABG) for multiple vessels was attempted, but the blood flow in the bilateral internal thoracic and gastroepiploic arteries was to poor for a donor artery, and the calcification of the ascending aortic wall was too severe for anastomosis of saphenous vein grafts. Therefore, the proper hepatic artery was connected to the left anterior descending artery using a vein graft. In April 2000, the patient's angina worsened. Occlusions of both subclavian arteries, bilateral coronary ostial stenosis and vein graft occlusion, aortic valve regurgitation, and two severe stenoses of the descending aorta were observed. Aortic valve replacement, and coronary and aorta revascularization were desirable, but the severe aortic wall calcification and thickening rendered these interventions impossible. Treatment with medication was chosen. The patient was discharged without severe angina. A combination of these serious cardiovascular complications which do not allow any surgical intervention is very rare. Received: May 21, 2001 / Accepted: August 24, 2001