Takayasu arteritis (TA) primarily causes ischaemic nephrosclerosis but can occasionally be associated with glomerulopathy. We report a case of a female in her twenties with PLA2-negative, THSD7A-positive membranous nephropathy (MN) refractory to rituximab, who presented with neck pain and new-onset hypertension. Blood work showed elevated inflammatory markers. Imaging of the head and neck revealed focal dilation and irregularity of the vertebral arteries, consistent with TA. The patient was started on treatment with steroids, followed by mycophenolate mofetil, which led to the resolution of symptoms and nephrotic syndrome. This case highlights an uncommon sequence of events, with MN presenting before TA, underscoring the need to consider TA in differentials for patients with MN. Notably, this is the first reported case in a young female, emphasising the need for further understanding of TA-associated glomerular diseases. Additionally, the presence of THSD7A in MN, despite negative malignancy workup, is also noteworthy.
CONCLUSIONS: Membranous nephropathy (MN) and Takayasu arteritis (TA) have distinct clinical presentations; therefore, diagnosing coexisting MN and TA is challenging, which may lead to delayed diagnoses.A multidisciplinary approach with tailored treatments is essential for prompt diagnosis and optimal management.Comprehensive follow-up studies are vital to understand the pathogenesis of this rare amalgamation, refine targeted treatment strategies and potentially improve overall prognosis.

BACKGROUND: Takayasu arteritis (TA) is a chronic inflammatory disease of unknown etiology characterized by a large vessel vasculitis involving the aorta and its branches. Myocardial involvement is extremely unusual in TA and is mostly in the form of myocarditis, ventricular hypertrophy, and ventricular dysfunction secondary to coronary ischemia. Submitral aneurysms have been reported in TA and has been attributed to the chronic inflammatory process in TA.
METHODS: We report a novel instance of left ventricular apical aneurysm in a 37-year-old lady with TA and normal epicardial coronaries. She was diagnosed with a left ventricular apical aneurysm, moderate aortic regurgitation, and moderate pericardial effusion. The coronary arteries were normal. The patient had concomitant chronic active Epstein-Barr virus infection complicating patient outcome.
CONCLUSIONS: Left ventricular apical aneurysm with normal epicardial coronaries is a rare cause of heart failure in Takayasu arteritis. Concomitant chronic active Epstein-Barr virus infection can potentially accentuate the inflammatory process in Takayasu arteritis and complicate management and patient outcomes.

OBJECTIVE: This study aimed to understand the status quo of medical treatments of the primary disease and pregnancy outcomes in patients with Takayasu arteritis (TAK) and children\'s birth outcomes.
METHODS: This study retrospectively enrolled patients with TAK who conceived after the disease onset and were managed at medical facilities participating in the Japan Research Committee of the Ministry of Health, Labor, and Welfare for Intractable Vasculitis.
RESULTS: This study enrolled 51 cases and 68 pregnancies 2019-2021. Of these, 48 cases and 65 pregnancies (95.6%) resulted in delivery and live-born babies. The median age of diagnosis and delivery was 22 and 31, respectively. Preconception therapy included prednisolone (PSL) in 51 (78.5%, median 7.5 mg/day), immunosuppressants in 18 (27.7%), and biologics in 12 (18.5%) pregnancies. Six cases underwent surgical treatment before pregnancy. Medications during pregnancy included PSL in 48 (73.8%, median: 9 mg/day), immunosuppressants in 13 (20.0%), and biologics in 9 (13.8%) pregnancies. Enlargement of an aneurysm was reported in one pregnancy, which might be associated with increased circulating plasma volume. TAK relapsed in 4 (6.2%) and 8 (12.3%) pregnancies during pregnancy and after delivery, respectively. Additionally, 13/62 (20.9%) preterm infants and 17/59 (28.8%) low birth weight infants were observed, and none had serious postnatal abnormalities. Of the 51 confirmed infants, 42 (82.4%) were exclusively breastfed or mixed with formula.
CONCLUSIONS: Most pregnancies in TAK were manageable with PSL at ≤10 mg/day. Relapse during pregnancy and postpartum occurred in <20% of pregnancies.

UNASSIGNED: Takayasu arteritis is a large-vessel vasculitis that affects the aorta and its primary branches. Myocarditis is a rare life-threatening complication and potential diagnostic pitfall in patients with Takayasu arteritis.
UNASSIGNED: A previously healthy 18-year-old woman presenting with fever, back pain, and dyspnoea was admitted to another hospital for acute hypertension (blood pressure, 230/106 mmHg) and congestive heart failure. Intravenous methylprednisolone pulse with antihypertensive and diuretic medications slightly improved her congestion. However, she developed acute kidney injury and was transferred to our hospital. Transthoracic echocardiography indicated a left ventricular ejection fraction of 45% and diffuse left ventricular hypokinesis. Doppler ultrasound test and magnetic resonance angiography revealed severe bilateral renal artery stenosis. Her diagnosis was Takayasu arteritis, and she received high-dose glucocorticoids. She required temporary haemodialysis, but 2 months after admission, her serum creatinine improved to 1.1 mg/dL without surgical or cardiovascular interventions. Although the pre-discharge test with 1.5 T cardiac magnetic resonance initially failed to diagnose myocarditis, 3 T cardiac magnetic resonance imaging revealed increased native T1 values on T1 mapping (1283-1393 ms), moderate pericardial effusion, and systolic left ventricular wall motion abnormality, indicating active myocarditis. During 6-month subcutaneous tocilizumab treatment (162 mg/week), a left ventricular ejection fraction improved to 55-60% without a relapse.
UNASSIGNED: This case report highlights the benefits of early multimodal imaging tests including cardiac magnetic resonance imaging for myocarditis and renal artery involvement in Takayasu arteritis. Tocilizumab might be an efficient therapeutic option for severe acute manifestations including myocarditis in young women of reproductive age.

OBJECTIVE: Recently, a joint group of the American College of Rheumatology (ACR) and the European Alliance of Associations for Rheumatology (EULAR) proposed new criteria for Takayasu arteritis (TAK) (the 2022 ACR/EULAR criteria). This study applied the 2022 ACR/EULAR criteria to patients with previously diagnosed TAK based on the 1990 ACR criteria and investigated the concordance rate between the two criteria according to the four imaging modalities.
METHODS: This study reviewed the medical records of 179 patients who met the 1990 ACR criteria for TAK. The imaging modalities included conventional angiography, computed tomography angiography, fluorodeoxyglucose-positron emission tomography, and magnetic resonance angiography.
RESULTS: Regardless of the imaging modalities, the concordance rate between the two criteria was 85.5% when including all patients, whereas it increased to 98.1% when only patients aged ≤60 years were included. Among the four imaging modalities, computed tomography angiography exhibited the highest concordance rate between the two criteria (85.6%). The concordance rate among patients aged >60 years was 95.7%. Only one patient aged 50-60 years was reclassified as having both TAK and giant cell arteritis.
CONCLUSIONS: The concordance rate was 85.5% regardless of the imaging modalities and increased to 86.9% on simultaneous computed tomography angiography and fluorodeoxyglucose-positron emission tomography imaging.

UNASSIGNED: T-wave inversions on electrocardiograms (ECGs) indicate a variety of conditions, such as coronary artery disease, myocarditis, and cardiomyopathy. Pulmonary artery stenosis (PAS) and pulmonary hypertension (PH) may cause right ventricular enlargement and ischaemia, which are reflected as T-wave inversions on ECGs. Continuous ECG monitoring is crucial for detecting dynamic changes indicative of PAS progression and reversal in right heart remodelling.
UNASSIGNED: This report presents the case of a young woman who experienced exertional dyspnoea for 5 years with ECG findings showing T-wave inversions across multiple leads. The patient was diagnosed with PAS and PH caused by Takayasu arteritis (TA). Following three successful balloon pulmonary angioplasty sessions, the patient exhibited significant clinical improvement, including the remission of PAS and PH. Throughout a 59-month cumulative follow-up period, the sustained effectiveness of the treatment was evidenced by the regression of right heart remodelling, as manifested in the normalization of the initially inverted T-waves on the ECG.
UNASSIGNED: Electrocardiogram changes, including right axis deviation, right bundle branch block, a deep S wave in lead I (R/S < 1), and a prominent R wave in lead aVR (R/Q > 1), have been termed PAS syndrome, often linked to TA-associated PAS, especially in young East Asian females. Early diagnosis is crucial but challenging due to atypical symptoms. The non-invasive ECG is vital for detection, with balloon pulmonary angioplasty serving as an effective treatment for TA-induced PAS when surgery is not an option, improving outcomes and potentially reversing right heart remodelling.

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Aortitis is a rare disease entity of unknown prevalence. Primary aortitis mainly affects the thoracic aorta. They are most often diagnosed on imaging by grade III 18-FDG uptake of the aortic wall on PET, or by circumferential thickening>2.2mm on CT or MRI with late-stage contrast. More rarely, aortitis is histologically proven, as in some cases of clinically isolated aortitis discovered after planned aortic aneurysm surgery or during aortic dissection surgery. The most common histological types are granulomatous/giant cell or lymphoplasmacytic. Clinical signs associated with aortitis are often non-specific: asthenia, fever, dry cough, chest, back, lumbar or abdominal pain. Aortitis can be divided into different etiological categories: primary aortitis, which includes vasculitis with a preferential or exclusive tropism for the aortic wall, aortitis secondary to systemic or iatrogenic diseases, and infectious aortitis. The main etiologies of primary aortitis are giant cell arteritis (GCA), Takayasu arteritis (TA) or clinically isolated aortitis. Aortitis secondary to systemic diseases is seen in atrophying polychondritis, systemic lupus and inflammatory rheumatic diseases such as spondyloarthropathy and rheumatoid arthritis. In both ACG and AT, aortitis is a negative factor, characterized by a higher risk of relapse, cardiovascular complications and increased mortality. The management of aortitis is insufficiently codified, and relies on the control of cardiovascular risk factors, with particular monitoring of blood pressure and LDL cholesterol, and on corticosteroid therapy and immunosuppressive drugs, the use of which will depend on the disease associated with the aortitis, the initial severity and comorbidities.

Takayasu arteriitis (TA) is a rare systemic vasculitis, affecting large vessels, cardiac valves and myocardium. Cardiac involvement is a major cause of morbidity and mortality in such patients. This publication presents a clinical case of a patient with severe aortic regurgitation combined with restrictive cardiomyopathy. It is emphasized that surgical treatment is associated with potential difficulties in patients with TA due to its inflammatory nature, disease activity and multiorgan involvement.

Takayasu arteritis is a chronic inflammatory vasculitis with granulomatous panarteritis particularly impacting large vessels including the aorta and its branches, especially the subclavian arteries, with clinical manifestation dependent on the involved artery. Sequelae of the active disease vary, including stenosis, occlusions, or aneurysmal dilatations of the large vessels. The prevalence of Takayasu arteritis is higher in the Asian population and in Japan, but quite low in the United States, varying from 0.9-8.4 per million people. Ocular manifestations are rare and lead to a delay in diagnosis and appropriate treatment. Ocular manifestations include Takayasu retinopathy, anterior ischemic optic neuropathy (AION), retinal artery occlusion (RAO) and retinal vein occlusion (RVO). We present two cases in which central retinal artery occlusion (CRAO) was associated with Takayasu arteritis. CRAO is an ophthalmic emergency with an incidence of 1.9 per 100,000 person years in the United States; only 5% of cases are arteritic, which can be observed with inflammatory vasculitides secondary to the formation of immune deposits.