RARE DISEASE
Takayasu arteritis
Takayasu arteritis
Takayasu arteritis
Drug discovery
2
drugs
With orphan designations
Overview
Takayasu arteritis (TAK) is a rare, chronic large-vessel vasculitis characterized by autoimmune-mediated inflammation of the aorta and its primary branches, leading to stenosis, occlusion, or aneurysm formation [1][6][12]. It predominantly affects females under 40, with a 9:1 female-to-male ratio, and has higher prevalence in Asian populations [6][12][15]. Diagnosis relies on imaging (angiography, MRI) due to nonspecific early symptoms like fever and fatigue, followed by vascular complications such as hypertension, stroke, and limb ischemia [1][3][12].
Burden
Morbidity: Hypertension (56%), renal artery stenosis (28%), aortic insufficiency (19%), and stroke risk [4][7][9].
Healthcare utilization: 7.35-day average hospitalization (2.1 days longer than non-TAK patients) and increased costs [4][9][16].
Chronicity: Relapse rates exceed 40%, necessitating long-term immunosuppression and monitoring [8][11][18].
Therapies
First-line: High-dose corticosteroids (e.g., prednisone) for inflammation control [3][8][11].
Steroid-sparing agents: Methotrexate, azathioprine, or mycophenolate mofetil; biologics (tocilizumab, TNF inhibitors) for refractory disease [8][11][18].
Surgical interventions: Revascularization or endovascular procedures for critical stenosis/aneurysms [3][9][18].
Categories: rare circulatory system diseases, rare renal diseases, rare systemic and rheumatological diseases, rare systemic or rheumatologic diseases of childhood, rare transplant-related disorders
Research Papers
1,312 drug discovery papers about Takayasu arteritis, with 1 first-in-class and 1 next-in-class emerging drug candidates forecasted to outperform the average preclinical success rate. Recent publications:
1,312 drug discovery papers about Takayasu arteritis, with 1 first-in-class and 1 next-in-class emerging drug candidates forecasted to outperform the average preclinical success rate. Recent publications:
categories:
Small molecules
small molecules
2026-08-10 | Bilateral Ocular ischemia in a patient with Takayasu arteritis.
To describe a rare case of bilateral ocular ischemia as the initial presentation of Takayasu arteritis and highlight the role of early recognition and treatment in preventing irreversible vision loss. A woman in her 50s presented with a gradual, progressive diminution of vision, initially in the left eye and later involving the right. Comprehensive ophthalmic evaluation, systemic examination, fundus fluorescein angiography, and CT angiography of the head and neck were performed. Laboratory investigations, including ESR, CRP, and ANA, were obtained. The American College of Rheumatology (ACR) criteria were applied to support the diagnosis. The patient demonstrated severe ocular ischemic features, including attenuated retinal vessels, disc neovascularization, and intumescent cataract in the left eye. Systemic examination revealed feeble brachial and radial pulses with a significant inter-arm blood pressure difference. CT angiography showed narrowing of the right common carotid artery and non-opacification of both subclavian arteries. Laboratory findings revealed elevated ESR and CRP, with a positive ANA. Based on clinical, radiologic, and laboratory features, a diagnosis of bilateral ocular ischemia secondary to Takayasu arteritis was made. The patient was treated with oral corticosteroids (60 mg/day, tapered) and azathioprine. At 4 weeks, visual acuity improved to 6/36 in the right eye, while no improvement was noted in the left eye. Our case highlights bilateral ocular ischemia as an uncommon but vision-threatening manifestation of Takayasu arteritis. Ocular findings such as retinal vascular attenuation, disc neovascularization, delayed choroidal filling, and peripheral capillary non-perfusion may precede the diagnosis of systemic vasculitis. Recognition of associated systemic signs, including pulse deficits and inter-arm blood pressure discrepancies, facilitated timely diagnosis. Prompt initiation of corticosteroid and immunosuppressive therapy led to visual improvement in the right eye, underscoring the importance of early diagnosis and multidisciplinary management to prevent irreversible visual loss and systemic vascular complications. Bilateral ocular ischemia may be the first manifestation of Takayasu arteritis. This case underscores the importance of a high index of suspicion in patients with unexplained ischemic ocular features. Early systemic evaluation and prompt initiation of immunosuppressive therapy are essential to preserve vision and prevent further vascular complications.
2026-08-04 | Age-associated B cells contribute to inflammation via antibody-independent mechanisms in Takayasu's arteritis.
Although Takayasu's arteritis (TAK) is not a prototypical autoantibody-mediated disease, accumulating evidence suggests that B cells are involved. This study aimed to investigate the pathway of B-cell activation and its contributions to TAK pathogenesis. Histological analysis of paravascular lymph nodes and affected arteries was conducted to investigate B-cell activation pathways in TAK. Bulk RNA-seq, single-cell RNA-seq (scRNA-seq), flow cytometry, and in vitro experiments were performed to characterize the composition, transcriptomic features and functional profiles of B cells. The numeric and phenotypic alterations induced by TNF and JAK inhibition were assessed both in vitro and in 4 patients with TAK. Histological (n=5), flow cytometric (n=125) and bulk RNA-seq (n=12) analyses indicated the presence of extrafollicular response and upregulated age-associated B cell (ABC) production in TAK, along with cross-dataset transcriptomic differences between B cells from patients with TAK and systemic lupus erythematosus (SLE). Cross-dataset scRNA-seq analysis and in vitro experiments (n=5) demonstrated that ABC differentiation in TAK was largely uncoupled from antibody-secreting cell (ASC) generation, unlike that in SLE. Functional experiments showed that ABCs exhibited proinflammatory properties, including proinflammatory cytokine production, and that CD11c+ B cells, which contain the ABC compartment, promoted Th17 cell differentiation (n=6). In vitro, tofacitinib was more effective than adalimumab at reducing ABC proportions and attenuating their proinflammatory phenotype (n=15), consistent with trends in the exploratory clinical follow-up (n=4). ABCs promote inflammation through proinflammatory functions independent of ASC differentiation in TAK. JAK inhibition exerts distinct suppressive effects on ABCs compared with anti-TNF therapy.
2026-08-01 | TAKAYASU ARTERITIS PRESENTING AS MULTIFOCAL ACUTE INFARCTS IN A YOUNG FEMALE: A CASE REPORT
Takayasu arteritis (TA) is a rare, chronic granulomatous large-vessel vasculitis that involves the aorta and its major branches and predominantly affects young women. We report a 30-year-old woman with a six-month history of right upper-limb claudication who presented with sudden-onset altered sensorium following a one-day febrile illness. Clinical examination revealed bilateral carotid bruits with absent right upper-limb pulses and preserved lower-limb blood pressure. MRI brain demonstrated acute infarcts in the left fronto-temporal region and right caudate nucleus, and CT aortogram showed circumferential wall thickening with significant luminal narrowing of both common carotid arteries, confirming the diagnosis of Takayasu arteritis. The patient was managed with high-dose oral corticosteroids and antiplatelet therapy, with endovascular stenting of the right common carotid artery planned. This case highlights the importance of considering TA in young women presenting with stroke and limb claudication, and the need for early immunosuppressive therapy to prevent irreversible vascular damage.
2026-07-29 | A Case of Elderly-Onset Ulcerative Colitis with Large-Vessel Giant Cell Arteritis Treated Successfully with Tofocitinib.
Large-vessel vasculitis, including Takayasu arteritis and giant cell arteritis (GCA), is a recognized extraintestinal manifestation of ulcerative colitis (UC), but its optimal management remains unclear. GCA is the principal systemic vasculitis in individuals aged over 50 years, and large-vessel involvement may occur with or without cranial disease. We report a case of UC complicated by inflammation of the thoracic aorta and the proximal left subclavian and brachiocephalic arteries. A 78-year-old man was diagnosed with proctitis-type UC. Mesalamine was switched to rectal budesonide enema plus azathioprine owing to intolerance. One month later, fever and neck pain appeared. Continuous linear hyperintensity on T2-weighted magnetic resonance imaging extended from part of the ascending aorta through the descending aorta, involving the aortic arch and the proximal left subclavian and brachiocephalic arteries and becoming less conspicuous distally. Corresponding images of contrast-enhanced T1-weighted sampling perfection with application-optimized contrasts using different flip-angle evolutions with spectral pre-saturation with inversion recovery demonstrated matching mural enhancement, consistent with large-vessel GCA. Tocilizumab was administered, but UC relapsed, prompting adalimumab initiation, which later resulted in loss of response. Infliximab was attempted but was discontinued because of an infusion reaction. Prednisolone 10 mg/day resulted in improvement, and tofacitinib was introduced for steroid tapering. This case highlights the importance of considering large-vessel GCA in elderly-onset UC with unexplained fever and demonstrates the potential role of tofacitinib in achieving steroid-free remission and vascular inflammation, offering a potential novel therapeutic option for refractory overlap syndromes.
2026-07-22 | 2025 EULAR recommendations for the management of polymyalgia rheumatica and primary large vessel vasculitis.
This study aimed to update the European Alliance of Associations for Rheumatology (EULAR) recommendations for management of polymyalgia rheumatica (PMR), giant cell arteritis (GCA), and Takayasu arteritis (TAK). Following EULAR standard operating procedures, systematic literature reviews identified data published since the previous versions for PMR (2015) and for GCA/TAK (2018). An international task force discussed the evidence, and following a structured voting process, created overarching principles, recommendations, and quality indicators. The task force agreed on 4 overarching principles, 12 recommendations, and 2 quality indicators. All patients with suspected PMR, GCA, or TAK should be referred for specialist assessment and those with suspected GCA within 24 hours. In patients with a strong clinical suspicion of GCA, treatment with glucocorticoids (GCs) should be commenced without delay, while waiting for the results of confirmatory investigations. For most patients with suspected PMR or TAK, treatment with GC can be delayed until the diagnosis is confirmed. For induction of remission, GC should be initiated at a dose of 15 to 25 mg/day prednisone-equivalent for PMR and 40 to 60 mg for GCA or TAK. Guidance on GC tapering is provided. Adjunctive therapy is recommended for selected patients with PMR or GCA using either interleukin (IL)-6 receptor inhibitors for PMR and GCA or upadacitinib for GCA. Methotrexate may be an alternative. GC-sparing agents should be given in combination with GC to all patients with TAK. All overarching principles and recommendations met with high levels of agreement. Updated guidance on management of PMR and large vessel vasculitis is provided in a single set of practical recommendations and algorithms.
cell therapies
2026-06-19 | Emergency renal autotransplantation for kidney salvage following iatrogenic renal artery occlusion after aortic stent deployment: A case report.
Renal autotransplantation (RAT) is an established reconstructive technique but is rarely used as an emergency salvage procedure. We report a 20-year-old male with Takayasu arteritis who developed acute left renal artery occlusion following endovascular stenting of infrarenal aortic stenosis. Imaging confirmed complete occlusion of the renal artery ostium due to stent coverage, precluding endovascular revascularization. Emergency nephrectomy and RAT to the right iliac fossa was performed. Postoperatively, renal function progressively recovered with stable graft perfusion on follow-up. This case highlights that RAT can be an effective salvage strategy in catastrophic renovascular complications when conventional endovascular rescue is not feasible.
2026-04-03 | Resection and patch aortoplasty for right renal artery aneurysm through a midline laparotomy.
BACKGROUND: Renal artery aneurysm (RAA) is a rare but potentially life-threatening vascular pathology. Surgical management depends on the aneurysm’s location and morphology. In cases with extensive aortic calcification or anatomical complexity, standard clamping techniques may be infeasible, requiring alternative hemostatic strategies. We present a unique case of RAA in a patient with Takayasu’s arteritis and diffuse aortic calcification, in whom intra-aortic balloon occlusion and patch aortoplasty were used as a salvage technique during surgical repair. CASE PRESENTATION: An 80-year-old woman with a history of Takayasu’s arteritis was referred for surgical treatment of an enlarging right RAA (55 × 74 mm), incidentally detected on imaging during hospitalization for pneumonia. Preoperative computed tomography (CT) demonstrated diffuse calcification of the entire aorta and an aneurysm located at the origin of the right renal artery. The distal segment of the right renal artery was occluded, and the right kidney could not be identified. Due to anticipated bleeding risk and poor clamping accessibility, cardiopulmonary bypass was established via the right femoral vessels. Multiple intra-aortic balloon occlusion catheters were inserted, though several ruptured intraoperatively. Hemostasis was ultimately achieved by inflating a balloon inserted directly through the operative field and performing a patch closure of the dilated right renal artery ostium. The patient required massive transfusion and temporary renal replacement therapy postoperatively. She was discharged to rehabilitation on postoperative day 49. Postoperative CT confirmed patch integrity, but a localized aortic dissection at the balloon site was observed. CONCLUSIONS: This case illustrated the technical challenges of surgical approaches for RAA in patients with severe aortic calcification due to Takayasu’s arteritis. Intra-aortic balloon occlusion is a useful alternative to conventional clamping but carries a risk of rupture, particularly in calcified aorta. Patch aortoplasty may be considered for elderly patients where graft replacement is not feasible. Preoperative imaging is essential to plan individualized strategies for bleeding control and vascular access in high-risk patients.
2026-03-27 | Ischemic Stroke as the First Manifestation of Takayasu Arteritis: A Case Report.
Ischemic stroke in young adults is uncommon and is frequently associated with rare etiologies, including autoimmune diseases and vasculitis. Takayasu arteritis (TA) is a chronic inflammatory large-vessel arteriopathy involving the aorta and its major branches and may result in cerebral ischemia due to arterial stenosis or thrombosis. We report the case of a 26-year-old woman with a history of suspected rheumatoid arthritis and Lyme disease who presented with acute left-sided hemiparesis and dysarthria. At admission, large-vessel vasculitis had not yet been suspected, and the patient was treated according to standard acute stroke protocols. Computed tomography angiography (CTA) revealed occlusion of the right middle cerebral artery bifurcation and the right common carotid artery, with inflammatory changes involving the brachiocephalic trunk and subclavian arteries. Intravenous thrombolysis (iv rtPA) was followed by mechanical thrombectomy (MT), resulting in neurological improvement. Further diagnostic work-up confirmed TA, and immunosuppressive therapy with cyclophosphamide and infliximab was initiated. This case underscores the importance of considering inflammatory large-vessel disease in young patients presenting with acute ischemic stroke and illustrates that endovascular reperfusion may be feasible in this clinical setting.
2026-02-23 | Stent insertion for chronic total occlusion of the common carotid artery in a patient with Takayasu arteritis using an adjunctive retrograde approach via superficial temporal artery puncture: illustrative case.
Takayasu arteritis is a large-vessel vasculitis characterized by chronic inflammatory lesions that frequently lead to stenosis and occlusion of the common carotid artery (CCA). Endovascular revascularization for these inflammatory lesions is particularly challenging because the fibrotic and calcified vessel walls frequently hinder the passage of wires and catheters from a conventional antegrade approach. The authors report the case of a 57-year-old female with Takayasu arteritis who presented with a chronic total occlusion of the right CCA. The initial antegrade endovascular attempt resulted in wire dissection, necessitating an alternative strategy. They successfully performed retrograde recanalization via surgical cutdown of the superficial temporal artery (STA). A pull-through technique was utilized to establish a wire rail, which enabled safe and effective placement of the stent across the occluded segment. The patient's postoperative course was uneventful. The present case illustrates that a retrograde approach from the STA is a viable and effective alternative strategy for treating challenging CCA occlusions in patients with Takayasu arteritis. This technique expands the therapeutic options for complex vascular lesions that might otherwise be considered untreatable using conventional methods. https://thejns.org/doi/10.3171/CASE25897.
2025-09-11 | Navigating complex clinical decisions: kidney transplantation following abdominal aorto-aortic bypass in infantile Takayasu arteritis.
Takayasu arteritis (TAK) is a granulomatous large-vessel vasculitis typically affecting young adult females. Pediatric cases are rare, and infantile onset is exceptional. Management relies on immunosuppression, with surgery reserved for severe complications. We describe a now 5.5-year-old boy diagnosed with TAK at six months of age, presenting with hypertensive encephalopathy and kidney dysfunction. Despite treatment with corticosteroids and anti-TNFα, his kidney function deteriorated, leading to kidney failure and dialysis. At nearly three years of age, he underwent abdominal aorto-aortic bypass and bilateral nephrectomy due to progressive vascular narrowing and refractory hypertension. At age four, he successfully received a deceased-donor kidney transplant. Eighteen months post-transplant, he maintains excellent graft function and shows no signs of TAK recurrence. This case underscores the complexity of diagnosing and managing infantile TAK with multiorgan involvement. To our knowledge, he is among the youngest reported TAK patients to undergo successful kidney transplantation following major vascular surgery. His course demonstrates the potential for long-term remission and safe transplantation under standard immunosuppression, without continued anti-TNFα therapy. The literature is sparse regarding kidney failure and transplantation in TAK, particularly in infants. This case highlights key management dilemmas in infantile TAK, including clinical diagnosis, timing of surgery and transplantation, choice of immunosuppression, and long-term monitoring. It emphasizes the importance of a multidisciplinary approach and the need for collaborative research to address knowledge gaps in this rare but complex condition.
proteins
2025-06-09 | The role of granulocyte colony-stimulating factor in Takayasu arteritis.
Previous studies have suggested that granulocyte colony-stimulating factor (G-CSF) treatment may trigger large vessel vasculitis (LVV). However, the role of G-CSF in Takayasu arteritis (TAK), a form of LVV, remains unclear. This study aims to investigate the potential role of G-CSF in TAK. This cross-sectional study included 40 TAK patients who met the 1990 American College of Rheumatology (ACR) classification criteria for Takayasu arteritis and 27 healthy controls (HCs). Group differences and correlations between G-CSF level, clinical parameters, and cytokine profiles were analyzed using appropriate tests. Aortic tissues from TAK patients were also examined for G-CSF expression using immunohistochemistry. G-CSF levels were significantly higher in TAK patients compared to controls (p < 0.0001), but no difference was found between active and inactive TAK. G-CSF levels positively correlated with neutrophil (R = 0.350, p = 0.028) and platelet counts (R = 0.466, p = 0.005). Elevated G-CSF levels were associated with several cytokines, including IL-6 (R = 0.672, p < 0.001), IL-17 (R = 0.692, p < 0.001), and IFN-γ (R = 0.897, p < 0.001). G-CSF may contribute to the inflammatory process and pathogenesis of TAK, offering new perspectives for its diagnosis and treatment. Key Points • This study systematically evaluated the role of G-CSF in TAK. • G-CSF level was significantly higher in TAK patients. • G-CSF level was found to have a significant positive correlation with neutrophil and platelet counts, as well as several cytokines in TAK patients. • G-CSF may contribute to the TAK process through the PI3 K-Akt pathway.
2024-12-16 | Transcatheter thrombolysis in children with Takayasu's arteritis complicated by acute cerebral infarction: a case report.
Takayasu arteritis (TA), a rare systemic macrovasculitis in children, affects multiple arteries, causing necrosis, remodeling, stenosis, or dilatation. Due to nonspecific symptoms, early diagnosis is challenging, and it is often missed until organ dysfunction occurs. TA poses a significant health threat, with few reports on acute cerebral infarction in pediatric cases. An 11-year-old girl presented with TA-induced thrombosis in the right middle cerebral artery's upper trunk. Despite prompt thrombolysis restoring blood flow, brain tissue necrosis and dysfunction persisted. Pre-intervention MRA showed occlusion and stenosis in relevant arteries. Subsequent angiography confirmed multiple-artery acute stroke, supported by imaging, serology, and cerebrospinal fluid tests. Timely rehabilitation, hormone treatment, and early diagnosis led to a favorable outcome. Early diagnosis and treatment of pediatric TA are crucial to prevent organ damage. Transcatheter thrombolysis effectively restores brain perfusion in acute infarction cases.
2022-12-14 | Regulatory T cells and systemic vasculitis.
Emerging data suggest that regulatory T-cells (Treg) alterations play a major role in systemic vasculitis pathophysiology. We performed a systematic review of recent advances in the role of Treg and interleukin (IL)-10 in the pathogenesis and treatment of systemic vasculitis, including giant cell arteritis (GCA), Takayasu arteritis, Behçet's disease, antineutrophil cytoplasm antibodies (ANCA) associated vasculitis (AAV), and cryoglobulinemia associated vasculitis. Emerging data suggest that Treg deficiencies are disease-specific, affecting distinct pathways in distinct vasculitides. Decreased peripheral blood frequencies of Treg are described in all vasculitis when compared to healthy donors. Altered Treg functions are reported in GCA, Takayasu arteritis, AAV, and Behçet's disease with different mechanisms proposed. Treatment with biologics, and sometimes other immunosuppressants, may restore Treg frequencies and/or immune activity with significant differences in active disease or disease in remission in several systemic vasculitis. IL-10 is elevated in GCA, AAV, cryoglobulinemia associated vasculitis. In Behçet's disease, IL-10 is decreased in peripheral blood and elevated in saliva. In Takayasu arteritis, IL-10 levels were essentially elevated in patients' vessel wall. Several new therapeutic approaches targeting Treg and Il-10 (low dose IL-2, CAR Treg…) are developed to treat patients with systemic vasculitis. Treg and IL-10 play a central role in the regulation of inflammation in vasculitis and new targeting approaches are emerging.
2021-09-20 | Bridging Therapy for Acute Stroke as the Initial Manifestation of Takayasu Arteritis: A Case Report and Review of Literature.
Takayasu arteritis (TA) is a chronic inflammatory disease involving the aorta and its principal branches. Acute ischaemic stroke (AIS) as the initial manifestation of TA is uncommon. There is little evidence on the efficacy of bridging therapy for AIS induced by TA. A 23-year-old Chinese woman with a suspected stroke presented to our hospital with sudden onset of right-sided weakness, right facial palsy, and aphasia that occurred 1 hour ago. After physical and ancillary examinations, recombinant tissue plasminogen activator was administered to the patient, which led to partial recovery. Her neurological function deteriorated with a large salvageable ischaemic penumbra on computed tomography perfusion. Cerebrovascular angiography showed multiple stenoses in the brachiocephalic trunk, the beginning of the right common carotid artery (CCA), and the bilateral subclavian arteries, as well as occlusion of the left CCA and its branches. Mechanical thrombectomy of the left middle cerebral artery was performed immediately. Percutaneous transluminal balloon angioplasty of the left CCA followed by stent implantation of the proximal left CCA was then performed. A diagnosis of TA was made based on the findings. The patient's neurological deficit fully recovered with immunosuppressants at the 3 month-follow-up. We report a rare case of a patient with TA initially presenting with AIS treated with bridging therapy with full recovery of neurological function. Bridging therapy should be taken into consideration for AIS in patients with TA. Further study is needed in this regard.
2013-05-17 | Takayasu's arteritis mimicking Kawasaki disease in 7-month-old infant, successfully treated with glucocorticosteroids and intravenous immunoglobulins.
Takayasu's arteritis (TA) rarely occurs in infants. There are only four reports on TA in children below 1 year of age, revealing diversity of its symptoms. We describe a 7-month-old female infant hospitalized for hectic fever, irritability, high acute phase indices, and coronary artery dilatations found on echocardiography, which suggested Kawasaki disease (KD). Despite of standard treatment, rapid development of thoracic and abdominal aorta aneurysms occurred, while coronary artery abnormalities regressed. The initial diagnosis was changed for TA. Subsequently implemented glucocorticosteroids led to clinical and laboratory normalization. However, 2 months after treatment discontinuation, TA relapsed. Glucocorticosteroid therapy was restarted with additional introduction of intravenous immunoglobulins (IVIG), resulting in full and stable remission lasting over 1 year. Thus, diagnosis and treatment of infant TA pose a big challenge to physicians. TA onset in infants may mimics KD. Prolonged glucocorticosteroids and IVIG administration can be recommended in the youngest patients with TA.
antibodies
2026-08-06 | Misty Mesentery as an Initial Imaging Finding Suggestive of Large Vessel Vasculitis.
Large vessel vasculitis (LVV) refers to vasculitis predominantly affecting large arteries, including the aorta and its major branches. It encompasses conditions such as giant cell arteritis and Takayasu arteritis, as well as unclassified or idiopathic forms. Imaging techniques, including computed tomography (CT), are essential for detecting large vessel involvement, particularly when biopsy findings are negative. We report the case of a 56-year-old man admitted with fever, headache, and upper abdominal pain lasting 2 weeks. On examination, he had scalp tenderness and upper abdominal tenderness without temporal artery enlargement. Laboratory tests revealed elevated C-reactive protein (CRP: 19.75 mg/dL) and normal IgG4 (45 mg/dL). Initial CT demonstrated increased attenuation of mesenteric fat (Figure 1), suggestive of misty mesentery. Antibiotic therapy for presumed infectious mesenteritis was ineffective, and CRP increased further to 30.20 mg/dL. Contrast-enhanced CT showed thickening of the aortic wall (Figure 2). Based on fever, headache, systemic inflammation, and aortic wall thickening, LVV was considered the most plausible diagnosis. Cite this article as: Noda S, Kondo S. Misty mesentery as an initial imaging finding suggestive of large vessel vasculitis. Eur J Rheumatol. 13(1), 0084, doi: 10.5152/eurjrheum.2026.25084.
2026-08-01 | Biological therapy in Takayasu arteritis. Results from the REVAS registry.
Biological therapy in patients with Takayasu arteritis is frequently used, but the factors associated with its use have not been determined. The present study aims to describe and identify predictors of the need for biological therapy in this vasculitis. We conducted a retrospective, multicentre, descriptive study including all patients with Takayasu arteritis enrolled in the REVAS registry from Spain. We performed a descriptive and a multivariate logistic regression analysis to identify variables associated with the need of biological therapy requirement. The study included 77 patients, 65 (84.4 %) women, with a median follow up of 9.9 (interquartile range 3.5-21.5) years. Sixty-three (81.8 %) participants received glucocorticoids and 45 (58.4 %) needed immunosuppressive therapy. Twenty-three patients had received biological therapy, 20 of whom were diagnosed after 2006. Biological therapy included infliximab (n = 3), adalimumab (n = 9) and tocilizumab (n = 18). Patients requiring biological therapy had a higher proportion of systemic symptoms (fever and/or constitutional syndrome) at baseline (70.0 %vs. 28.6 %, p = 0.005), received glucocorticoids more frequently (100.0 %vs. 78.6 %, p = 0.03) and required a greater number of immunosuppressants (2 (1-2) vs. 1 (0-1), p = 0.03) during follow-up. On multivariate logistic regression, the presence of systemic symptoms was associated with the need for biological therapy requirement (odds radio: 3.9 (1.02-14.8), p = 0.047). Conclusion: to our knowledge, this is the first study that evaluates variables associated with the need for biological therapy requirement in Takayasu arteritis. The presence of systemic symptoms at presentation was independently associated with the need for biological therapy, highlighting the importance of a thorough clinical evaluation.
2026-07-22 | Prognostic Value of the Hata Angiographic Classification for Long-Term Outcomes in Takayasu Arteritis: A Single-Center, Retrospective Study.
The prognostic utility of the Hata angiographic classification for long-term outcomes in Takayasu arteritis (TAK) remains unclear. To evaluate the prognostic value of the Hata classification for predicting major adverse cardiovascular events (MACE) and the need for vascular interventions in patients with TAK. This single-center, retrospective cohort study included patients diagnosed with TAK between 2000 and 2025. Multivariate logistic regression analyses were performed to identify independent predictors, with statistical significance defined as p < 0.05. Of 203 patients initially identified, 18 were excluded. The final cohort included 185 patients, of whom 80.0% were women, with a median age at diagnosis of 29 years. Type V was the predominant angiographic pattern (64.9%). During a median follow-up of 10.8 years, MACE occurred in 35.7% of patients, while 37.3% required vascular intervention. The Hata classification was not associated with MACE occurrence. Independent predictors of MACE included female sex, which was protective (odds ratio [OR], 0.38; 95% CI, 0.17-0.86; p = 0.021), and longer symptom onset-to-diagnosis time, which increased risk (OR, 1.10; 95% CI, 1.03-1.18; p = 0.006). In contrast, the Hata classification independently predicted the need for vascular intervention. Hata Type I showed a strong protective association compared with Type V (OR, 0.10; 95% CI, 0.01-0.53; p = 0.029). Baseline aortic insufficiency was also independently associated with a higher likelihood of intervention (OR, 2.54; 95% CI, 1.18-5.60; p = 0.018) (Central Illustration). The Hata classification appears to be a robust predictor of vascular intervention but not MACE in TAK. Greater anatomical disease extension (Type V) and baseline aortic insufficiency were associated with increased procedural risk, whereas MACE risk was associated with diagnostic delay and male sex. The observed long-term dissociation between high rates of clinical remission (92.4%) and continued disease progression underscores the importance of imaging-based surveillance and highlights the need for prospective multicenter studies to validate these findings.
2026-06-12 | Aortitis accelerates the growth rate of thoracic aortic aneurysms.
Thoracic aortic aneurysms (TAA) are predominantly degenerative. Non-infectious inflammatory aortitis, including giant cell arteritis (GCA), Takayasu arteritis and clinically isolated aortitis (CIA), represents a less understood aetiology with poorly characterised growth patterns. While GCA is linked to higher TAA risk, preoperative growth data for inflammatory TAA are scarce. This study compares growth rates in histologically proven inflammatory aortitis. We conducted a retrospective case-control study, including patients with histologically proven aortitis and matched controls with degenerative TAA. Cases and controls were matched for age, sex, surgical year and procedure type. Aneurysm growth rate (mm/month) was calculated from serial imaging. This study included 23 patients with histologically confirmed aortitis (13 GCA, 9 CIA) and 42 matched controls with degenerative TAA. The mean growth rate was significantly higher in the aortitis group (0.177±0.01 mm/month; 2.12 mm/year) compared with controls (0.039±0.02 mm/month; 0.47 mm/year), representing a 4.5-fold acceleration (p<0.001). Among inflammatory etiologies, CIA demonstrated faster growth (0.338±0.06 mm/month; 4.06 mm/year) than GCA (2.1 mm/year; p<0.01). Patients with CIA more frequently required Bentall procedures (33% vs 0%, p=0.047). No differences were observed in baseline demographics, cardiovascular risk factors or baseline aortic diameters between groups. Histologically, 70% of aortitis cases exhibited granulomatous inflammation. Inflammatory aortitis is associated with a 4.5-fold faster preoperative TAA growth rate compared with degenerative aneurysms, with CIA exhibiting two times the growth rate of GCA. Prospective studies are warranted to validate these results and optimise surveillance and management strategies for this high-risk population.
2026-06-06 | Incidental detection of Takayasu arteritis in a hypertensive pregnancy with recurrent pregnancy loss: a positive maternal outcome.
Takayasu arteritis, which is also known as aortic arch syndrome, non-specific aortoarteritis and pulseless disease, is a rare chronic inflammatory progressive large vessel vasculitis of likely autoimmune aetiology causing narrowing, occlusion and aneurysms of systemic and pulmonary arteries, especially the aorta and its branches, afflicting women of childbearing age. It is an uncommon disease with an estimated annual incidence rate of 1.2-2.6 per million. Hypertension is the the most common mode of presentation. Based on site and extent, it is divided into five types as type I (branches of aortic arch), type II (aortic arch, its branches and descending thoracic aorta), type III (descending thoracic aorta and abdominal aorta), type IV (abdominal aorta only) and type V (aortic arch, descending thoracic aorta and abdominal aorta). Therapeutic modalities include medical (antihypertensive, steroids, cyclophosphamide, etc) as well as surgical interventions.
other
2023-01-13 | [Analysis of differentially expressed microRNA and protein expression profiles carried by exosomes in the plasma of patients with Takayasu's arteritis].
Objective: To detect the microRNAs (miRNAs) and proteins carried by exosomes in the plasma of patients with newly diagnosed Takayasu's arteritis (TAK) and analyze their possible roles in the pathogenesis of TAK. Methods: Ten patients with newly diagnosed TAK from the Department of Rheumatology and Immunology, Peking Union Medical College Hospital were selected during June-November 2020. Five healthy controls were matched with five patients by age and sex. RNA sequencing and protein mass spectrometry were used to detect miRNAs and proteins, respectively, carried by exosomes in the plasma. Differentially expressed miRNAs (DE-miRNAs) and proteins (DEPs) were screened. Thereafter, hierarchical cluster analysis, function, signal pathway, and protein domain enrichment analysis of DE-miRNAs and DEPs were performed. Finally, miRNAs and proteins related to vasculitis and autoimmunity were identified. The possible roles of the miRNAs and proteins in the pathogenesis of TAK were explored. Enumeration data were compared using Fisher's exact probability test or Chi-square test, and a P-value<0.05 was considered significant. Results: Compared with the healthy controls, patients with TAK had 29 DE-miRNAs on their plasma exosomes. Among these DE-miRNAs, miR-101-3p, miR-122-5p, miR-143-3p, miR-185-3p, miR-192-5p, miR-194-5p, miR-19a-3p, miR-19b-3p, miR-20b-5p, miR-21-5p, miR-22-3p, miR-335-5p, miR-34a-5p, miR-3613-5p, miR-548ad-5p, miR-590-3p, and miR-7-5p were upregulated; whereas miR-1249-3p, miR-141-3p, miR-199a-5p, miR-199b-5p, miR-200a-3p, miR-200c-3p, miR-204-5p, miR-29c-5p, miR-335-3p, miR-381-3p, miR-4433b-5p, and miR-584-5p were downregulated. Finally, miR-34a-5p, miR-200c-3p, miR-143-3p, miR-22-3p, and miR-21-5p were identified. Among the 357 DEPs screened, 236 DEPs were upregulated, whereas 121 DEPs were downregulated. Finally, kallikrein B1 (KLKB1), kininogen 1 (KNG1), desmoplakin (DSP) were identified. Conclusion: MiR-34a-5p, miR-200c-3p, miR-143-3p, miR-22-3p, miR-21-5p, KLKB1, KNG1, and DSP carried by exosomes in plasma might participate in the pathogenesis of TAK by regulating vascular physiology, inflammation, autoimmunity, and other processes. They may be biomarkers and therapeutic targets of TAK. 目的： 检测初治多发性大动脉炎（TAK）患者血浆外泌体携带的微小RNA和蛋白，分析其在TAK发病机制中的可能作用。 方法： 选2020年6月至11月北京协和医院风湿免疫科确诊的初治TAK患者10例，另选与之年龄性别匹配的健康对照者5例，分别通过RNA测序和蛋白质谱技术检测血浆外泌体携带的微小RNA和蛋白，筛选出差异表达的微小RNA和蛋白，并对其进行层次聚类分析、功能、信号通路和结构域富集分析，最终筛选出与血管炎有关的微小RNA和蛋白，探究其在TAK发病机制中的可能作用。统计学采用Fisher精确概率检验或χ²检验。 结果： TAK患者血浆外泌体携带的差异表达的微小RNA 29个，其中miR-101-3p、miR-122-5p、miR-143-3p、miR-185-3p、miR-192-5p、miR-194-5p、miR-19a-3p、miR-19b-3p、miR-20b-5p、miR-21-5p、miR-22-3p、miR-335-5p、miR-34a-5p、miR-3613-5p、miR-548ad-5p、miR-590-3p、miR-7-5p表达上调，miR-1249-3p、miR-141-3p、miR-199a-5p、miR-199b-5p、miR-200a-3p、miR-200c-3p、miR-204-5p、miR-29c-5p、miR-335-3p、miR-381-3p、miR-4433b-5p、miR-584-5p表达下调。差异表达的蛋白357个，其中236个表达上调，121个表达下调，最终筛选前激肽释放酶、激肽原1、桥粒蛋白。 结论： 血浆外泌体携带的miR-34a-5p、miR-200c-3p、miR-143-3p、miR-22-3p、miR-21-5p、前激肽释放酶、激肽原1、桥粒蛋白可能通过调节血管生理过程、炎症反应、自身免疫等过程参与TAK的发病机制，具有作为TAK生物标志物和治疗靶点的潜在作用。.
small molecules
2026-08-10 | Bilateral Ocular ischemia in a patient with Takayasu arteritis.
To describe a rare case of bilateral ocular ischemia as the initial presentation of Takayasu arteritis and highlight the role of early recognition and treatment in preventing irreversible vision loss. A woman in her 50s presented with a gradual, progressive diminution of vision, initially in the left eye and later involving the right. Comprehensive ophthalmic evaluation, systemic examination, fundus fluorescein angiography, and CT angiography of the head and neck were performed. Laboratory investigations, including ESR, CRP, and ANA, were obtained. The American College of Rheumatology (ACR) criteria were applied to support the diagnosis. The patient demonstrated severe ocular ischemic features, including attenuated retinal vessels, disc neovascularization, and intumescent cataract in the left eye. Systemic examination revealed feeble brachial and radial pulses with a significant inter-arm blood pressure difference. CT angiography showed narrowing of the right common carotid artery and non-opacification of both subclavian arteries. Laboratory findings revealed elevated ESR and CRP, with a positive ANA. Based on clinical, radiologic, and laboratory features, a diagnosis of bilateral ocular ischemia secondary to Takayasu arteritis was made. The patient was treated with oral corticosteroids (60 mg/day, tapered) and azathioprine. At 4 weeks, visual acuity improved to 6/36 in the right eye, while no improvement was noted in the left eye. Our case highlights bilateral ocular ischemia as an uncommon but vision-threatening manifestation of Takayasu arteritis. Ocular findings such as retinal vascular attenuation, disc neovascularization, delayed choroidal filling, and peripheral capillary non-perfusion may precede the diagnosis of systemic vasculitis. Recognition of associated systemic signs, including pulse deficits and inter-arm blood pressure discrepancies, facilitated timely diagnosis. Prompt initiation of corticosteroid and immunosuppressive therapy led to visual improvement in the right eye, underscoring the importance of early diagnosis and multidisciplinary management to prevent irreversible visual loss and systemic vascular complications. Bilateral ocular ischemia may be the first manifestation of Takayasu arteritis. This case underscores the importance of a high index of suspicion in patients with unexplained ischemic ocular features. Early systemic evaluation and prompt initiation of immunosuppressive therapy are essential to preserve vision and prevent further vascular complications.
2026-08-04 | Age-associated B cells contribute to inflammation via antibody-independent mechanisms in Takayasu's arteritis.
Although Takayasu's arteritis (TAK) is not a prototypical autoantibody-mediated disease, accumulating evidence suggests that B cells are involved. This study aimed to investigate the pathway of B-cell activation and its contributions to TAK pathogenesis. Histological analysis of paravascular lymph nodes and affected arteries was conducted to investigate B-cell activation pathways in TAK. Bulk RNA-seq, single-cell RNA-seq (scRNA-seq), flow cytometry, and in vitro experiments were performed to characterize the composition, transcriptomic features and functional profiles of B cells. The numeric and phenotypic alterations induced by TNF and JAK inhibition were assessed both in vitro and in 4 patients with TAK. Histological (n=5), flow cytometric (n=125) and bulk RNA-seq (n=12) analyses indicated the presence of extrafollicular response and upregulated age-associated B cell (ABC) production in TAK, along with cross-dataset transcriptomic differences between B cells from patients with TAK and systemic lupus erythematosus (SLE). Cross-dataset scRNA-seq analysis and in vitro experiments (n=5) demonstrated that ABC differentiation in TAK was largely uncoupled from antibody-secreting cell (ASC) generation, unlike that in SLE. Functional experiments showed that ABCs exhibited proinflammatory properties, including proinflammatory cytokine production, and that CD11c+ B cells, which contain the ABC compartment, promoted Th17 cell differentiation (n=6). In vitro, tofacitinib was more effective than adalimumab at reducing ABC proportions and attenuating their proinflammatory phenotype (n=15), consistent with trends in the exploratory clinical follow-up (n=4). ABCs promote inflammation through proinflammatory functions independent of ASC differentiation in TAK. JAK inhibition exerts distinct suppressive effects on ABCs compared with anti-TNF therapy.
2026-08-01 | TAKAYASU ARTERITIS PRESENTING AS MULTIFOCAL ACUTE INFARCTS IN A YOUNG FEMALE: A CASE REPORT
Takayasu arteritis (TA) is a rare, chronic granulomatous large-vessel vasculitis that involves the aorta and its major branches and predominantly affects young women. We report a 30-year-old woman with a six-month history of right upper-limb claudication who presented with sudden-onset altered sensorium following a one-day febrile illness. Clinical examination revealed bilateral carotid bruits with absent right upper-limb pulses and preserved lower-limb blood pressure. MRI brain demonstrated acute infarcts in the left fronto-temporal region and right caudate nucleus, and CT aortogram showed circumferential wall thickening with significant luminal narrowing of both common carotid arteries, confirming the diagnosis of Takayasu arteritis. The patient was managed with high-dose oral corticosteroids and antiplatelet therapy, with endovascular stenting of the right common carotid artery planned. This case highlights the importance of considering TA in young women presenting with stroke and limb claudication, and the need for early immunosuppressive therapy to prevent irreversible vascular damage.
2026-07-29 | A Case of Elderly-Onset Ulcerative Colitis with Large-Vessel Giant Cell Arteritis Treated Successfully with Tofocitinib.
Large-vessel vasculitis, including Takayasu arteritis and giant cell arteritis (GCA), is a recognized extraintestinal manifestation of ulcerative colitis (UC), but its optimal management remains unclear. GCA is the principal systemic vasculitis in individuals aged over 50 years, and large-vessel involvement may occur with or without cranial disease. We report a case of UC complicated by inflammation of the thoracic aorta and the proximal left subclavian and brachiocephalic arteries. A 78-year-old man was diagnosed with proctitis-type UC. Mesalamine was switched to rectal budesonide enema plus azathioprine owing to intolerance. One month later, fever and neck pain appeared. Continuous linear hyperintensity on T2-weighted magnetic resonance imaging extended from part of the ascending aorta through the descending aorta, involving the aortic arch and the proximal left subclavian and brachiocephalic arteries and becoming less conspicuous distally. Corresponding images of contrast-enhanced T1-weighted sampling perfection with application-optimized contrasts using different flip-angle evolutions with spectral pre-saturation with inversion recovery demonstrated matching mural enhancement, consistent with large-vessel GCA. Tocilizumab was administered, but UC relapsed, prompting adalimumab initiation, which later resulted in loss of response. Infliximab was attempted but was discontinued because of an infusion reaction. Prednisolone 10 mg/day resulted in improvement, and tofacitinib was introduced for steroid tapering. This case highlights the importance of considering large-vessel GCA in elderly-onset UC with unexplained fever and demonstrates the potential role of tofacitinib in achieving steroid-free remission and vascular inflammation, offering a potential novel therapeutic option for refractory overlap syndromes.
2026-07-22 | 2025 EULAR recommendations for the management of polymyalgia rheumatica and primary large vessel vasculitis.
This study aimed to update the European Alliance of Associations for Rheumatology (EULAR) recommendations for management of polymyalgia rheumatica (PMR), giant cell arteritis (GCA), and Takayasu arteritis (TAK). Following EULAR standard operating procedures, systematic literature reviews identified data published since the previous versions for PMR (2015) and for GCA/TAK (2018). An international task force discussed the evidence, and following a structured voting process, created overarching principles, recommendations, and quality indicators. The task force agreed on 4 overarching principles, 12 recommendations, and 2 quality indicators. All patients with suspected PMR, GCA, or TAK should be referred for specialist assessment and those with suspected GCA within 24 hours. In patients with a strong clinical suspicion of GCA, treatment with glucocorticoids (GCs) should be commenced without delay, while waiting for the results of confirmatory investigations. For most patients with suspected PMR or TAK, treatment with GC can be delayed until the diagnosis is confirmed. For induction of remission, GC should be initiated at a dose of 15 to 25 mg/day prednisone-equivalent for PMR and 40 to 60 mg for GCA or TAK. Guidance on GC tapering is provided. Adjunctive therapy is recommended for selected patients with PMR or GCA using either interleukin (IL)-6 receptor inhibitors for PMR and GCA or upadacitinib for GCA. Methotrexate may be an alternative. GC-sparing agents should be given in combination with GC to all patients with TAK. All overarching principles and recommendations met with high levels of agreement. Updated guidance on management of PMR and large vessel vasculitis is provided in a single set of practical recommendations and algorithms.
cell therapies
2026-06-19 | Emergency renal autotransplantation for kidney salvage following iatrogenic renal artery occlusion after aortic stent deployment: A case report.
Renal autotransplantation (RAT) is an established reconstructive technique but is rarely used as an emergency salvage procedure. We report a 20-year-old male with Takayasu arteritis who developed acute left renal artery occlusion following endovascular stenting of infrarenal aortic stenosis. Imaging confirmed complete occlusion of the renal artery ostium due to stent coverage, precluding endovascular revascularization. Emergency nephrectomy and RAT to the right iliac fossa was performed. Postoperatively, renal function progressively recovered with stable graft perfusion on follow-up. This case highlights that RAT can be an effective salvage strategy in catastrophic renovascular complications when conventional endovascular rescue is not feasible.
2026-04-03 | Resection and patch aortoplasty for right renal artery aneurysm through a midline laparotomy.
BACKGROUND: Renal artery aneurysm (RAA) is a rare but potentially life-threatening vascular pathology. Surgical management depends on the aneurysm’s location and morphology. In cases with extensive aortic calcification or anatomical complexity, standard clamping techniques may be infeasible, requiring alternative hemostatic strategies. We present a unique case of RAA in a patient with Takayasu’s arteritis and diffuse aortic calcification, in whom intra-aortic balloon occlusion and patch aortoplasty were used as a salvage technique during surgical repair. CASE PRESENTATION: An 80-year-old woman with a history of Takayasu’s arteritis was referred for surgical treatment of an enlarging right RAA (55 × 74 mm), incidentally detected on imaging during hospitalization for pneumonia. Preoperative computed tomography (CT) demonstrated diffuse calcification of the entire aorta and an aneurysm located at the origin of the right renal artery. The distal segment of the right renal artery was occluded, and the right kidney could not be identified. Due to anticipated bleeding risk and poor clamping accessibility, cardiopulmonary bypass was established via the right femoral vessels. Multiple intra-aortic balloon occlusion catheters were inserted, though several ruptured intraoperatively. Hemostasis was ultimately achieved by inflating a balloon inserted directly through the operative field and performing a patch closure of the dilated right renal artery ostium. The patient required massive transfusion and temporary renal replacement therapy postoperatively. She was discharged to rehabilitation on postoperative day 49. Postoperative CT confirmed patch integrity, but a localized aortic dissection at the balloon site was observed. CONCLUSIONS: This case illustrated the technical challenges of surgical approaches for RAA in patients with severe aortic calcification due to Takayasu’s arteritis. Intra-aortic balloon occlusion is a useful alternative to conventional clamping but carries a risk of rupture, particularly in calcified aorta. Patch aortoplasty may be considered for elderly patients where graft replacement is not feasible. Preoperative imaging is essential to plan individualized strategies for bleeding control and vascular access in high-risk patients.
2026-03-27 | Ischemic Stroke as the First Manifestation of Takayasu Arteritis: A Case Report.
Ischemic stroke in young adults is uncommon and is frequently associated with rare etiologies, including autoimmune diseases and vasculitis. Takayasu arteritis (TA) is a chronic inflammatory large-vessel arteriopathy involving the aorta and its major branches and may result in cerebral ischemia due to arterial stenosis or thrombosis. We report the case of a 26-year-old woman with a history of suspected rheumatoid arthritis and Lyme disease who presented with acute left-sided hemiparesis and dysarthria. At admission, large-vessel vasculitis had not yet been suspected, and the patient was treated according to standard acute stroke protocols. Computed tomography angiography (CTA) revealed occlusion of the right middle cerebral artery bifurcation and the right common carotid artery, with inflammatory changes involving the brachiocephalic trunk and subclavian arteries. Intravenous thrombolysis (iv rtPA) was followed by mechanical thrombectomy (MT), resulting in neurological improvement. Further diagnostic work-up confirmed TA, and immunosuppressive therapy with cyclophosphamide and infliximab was initiated. This case underscores the importance of considering inflammatory large-vessel disease in young patients presenting with acute ischemic stroke and illustrates that endovascular reperfusion may be feasible in this clinical setting.
2026-02-23 | Stent insertion for chronic total occlusion of the common carotid artery in a patient with Takayasu arteritis using an adjunctive retrograde approach via superficial temporal artery puncture: illustrative case.
Takayasu arteritis is a large-vessel vasculitis characterized by chronic inflammatory lesions that frequently lead to stenosis and occlusion of the common carotid artery (CCA). Endovascular revascularization for these inflammatory lesions is particularly challenging because the fibrotic and calcified vessel walls frequently hinder the passage of wires and catheters from a conventional antegrade approach. The authors report the case of a 57-year-old female with Takayasu arteritis who presented with a chronic total occlusion of the right CCA. The initial antegrade endovascular attempt resulted in wire dissection, necessitating an alternative strategy. They successfully performed retrograde recanalization via surgical cutdown of the superficial temporal artery (STA). A pull-through technique was utilized to establish a wire rail, which enabled safe and effective placement of the stent across the occluded segment. The patient's postoperative course was uneventful. The present case illustrates that a retrograde approach from the STA is a viable and effective alternative strategy for treating challenging CCA occlusions in patients with Takayasu arteritis. This technique expands the therapeutic options for complex vascular lesions that might otherwise be considered untreatable using conventional methods. https://thejns.org/doi/10.3171/CASE25897.
2025-09-11 | Navigating complex clinical decisions: kidney transplantation following abdominal aorto-aortic bypass in infantile Takayasu arteritis.
Takayasu arteritis (TAK) is a granulomatous large-vessel vasculitis typically affecting young adult females. Pediatric cases are rare, and infantile onset is exceptional. Management relies on immunosuppression, with surgery reserved for severe complications. We describe a now 5.5-year-old boy diagnosed with TAK at six months of age, presenting with hypertensive encephalopathy and kidney dysfunction. Despite treatment with corticosteroids and anti-TNFα, his kidney function deteriorated, leading to kidney failure and dialysis. At nearly three years of age, he underwent abdominal aorto-aortic bypass and bilateral nephrectomy due to progressive vascular narrowing and refractory hypertension. At age four, he successfully received a deceased-donor kidney transplant. Eighteen months post-transplant, he maintains excellent graft function and shows no signs of TAK recurrence. This case underscores the complexity of diagnosing and managing infantile TAK with multiorgan involvement. To our knowledge, he is among the youngest reported TAK patients to undergo successful kidney transplantation following major vascular surgery. His course demonstrates the potential for long-term remission and safe transplantation under standard immunosuppression, without continued anti-TNFα therapy. The literature is sparse regarding kidney failure and transplantation in TAK, particularly in infants. This case highlights key management dilemmas in infantile TAK, including clinical diagnosis, timing of surgery and transplantation, choice of immunosuppression, and long-term monitoring. It emphasizes the importance of a multidisciplinary approach and the need for collaborative research to address knowledge gaps in this rare but complex condition.
proteins
2025-06-09 | The role of granulocyte colony-stimulating factor in Takayasu arteritis.
Previous studies have suggested that granulocyte colony-stimulating factor (G-CSF) treatment may trigger large vessel vasculitis (LVV). However, the role of G-CSF in Takayasu arteritis (TAK), a form of LVV, remains unclear. This study aims to investigate the potential role of G-CSF in TAK. This cross-sectional study included 40 TAK patients who met the 1990 American College of Rheumatology (ACR) classification criteria for Takayasu arteritis and 27 healthy controls (HCs). Group differences and correlations between G-CSF level, clinical parameters, and cytokine profiles were analyzed using appropriate tests. Aortic tissues from TAK patients were also examined for G-CSF expression using immunohistochemistry. G-CSF levels were significantly higher in TAK patients compared to controls (p < 0.0001), but no difference was found between active and inactive TAK. G-CSF levels positively correlated with neutrophil (R = 0.350, p = 0.028) and platelet counts (R = 0.466, p = 0.005). Elevated G-CSF levels were associated with several cytokines, including IL-6 (R = 0.672, p < 0.001), IL-17 (R = 0.692, p < 0.001), and IFN-γ (R = 0.897, p < 0.001). G-CSF may contribute to the inflammatory process and pathogenesis of TAK, offering new perspectives for its diagnosis and treatment. Key Points • This study systematically evaluated the role of G-CSF in TAK. • G-CSF level was significantly higher in TAK patients. • G-CSF level was found to have a significant positive correlation with neutrophil and platelet counts, as well as several cytokines in TAK patients. • G-CSF may contribute to the TAK process through the PI3 K-Akt pathway.
2024-12-16 | Transcatheter thrombolysis in children with Takayasu's arteritis complicated by acute cerebral infarction: a case report.
Takayasu arteritis (TA), a rare systemic macrovasculitis in children, affects multiple arteries, causing necrosis, remodeling, stenosis, or dilatation. Due to nonspecific symptoms, early diagnosis is challenging, and it is often missed until organ dysfunction occurs. TA poses a significant health threat, with few reports on acute cerebral infarction in pediatric cases. An 11-year-old girl presented with TA-induced thrombosis in the right middle cerebral artery's upper trunk. Despite prompt thrombolysis restoring blood flow, brain tissue necrosis and dysfunction persisted. Pre-intervention MRA showed occlusion and stenosis in relevant arteries. Subsequent angiography confirmed multiple-artery acute stroke, supported by imaging, serology, and cerebrospinal fluid tests. Timely rehabilitation, hormone treatment, and early diagnosis led to a favorable outcome. Early diagnosis and treatment of pediatric TA are crucial to prevent organ damage. Transcatheter thrombolysis effectively restores brain perfusion in acute infarction cases.
2022-12-14 | Regulatory T cells and systemic vasculitis.
Emerging data suggest that regulatory T-cells (Treg) alterations play a major role in systemic vasculitis pathophysiology. We performed a systematic review of recent advances in the role of Treg and interleukin (IL)-10 in the pathogenesis and treatment of systemic vasculitis, including giant cell arteritis (GCA), Takayasu arteritis, Behçet's disease, antineutrophil cytoplasm antibodies (ANCA) associated vasculitis (AAV), and cryoglobulinemia associated vasculitis. Emerging data suggest that Treg deficiencies are disease-specific, affecting distinct pathways in distinct vasculitides. Decreased peripheral blood frequencies of Treg are described in all vasculitis when compared to healthy donors. Altered Treg functions are reported in GCA, Takayasu arteritis, AAV, and Behçet's disease with different mechanisms proposed. Treatment with biologics, and sometimes other immunosuppressants, may restore Treg frequencies and/or immune activity with significant differences in active disease or disease in remission in several systemic vasculitis. IL-10 is elevated in GCA, AAV, cryoglobulinemia associated vasculitis. In Behçet's disease, IL-10 is decreased in peripheral blood and elevated in saliva. In Takayasu arteritis, IL-10 levels were essentially elevated in patients' vessel wall. Several new therapeutic approaches targeting Treg and Il-10 (low dose IL-2, CAR Treg…) are developed to treat patients with systemic vasculitis. Treg and IL-10 play a central role in the regulation of inflammation in vasculitis and new targeting approaches are emerging.
2021-09-20 | Bridging Therapy for Acute Stroke as the Initial Manifestation of Takayasu Arteritis: A Case Report and Review of Literature.
Takayasu arteritis (TA) is a chronic inflammatory disease involving the aorta and its principal branches. Acute ischaemic stroke (AIS) as the initial manifestation of TA is uncommon. There is little evidence on the efficacy of bridging therapy for AIS induced by TA. A 23-year-old Chinese woman with a suspected stroke presented to our hospital with sudden onset of right-sided weakness, right facial palsy, and aphasia that occurred 1 hour ago. After physical and ancillary examinations, recombinant tissue plasminogen activator was administered to the patient, which led to partial recovery. Her neurological function deteriorated with a large salvageable ischaemic penumbra on computed tomography perfusion. Cerebrovascular angiography showed multiple stenoses in the brachiocephalic trunk, the beginning of the right common carotid artery (CCA), and the bilateral subclavian arteries, as well as occlusion of the left CCA and its branches. Mechanical thrombectomy of the left middle cerebral artery was performed immediately. Percutaneous transluminal balloon angioplasty of the left CCA followed by stent implantation of the proximal left CCA was then performed. A diagnosis of TA was made based on the findings. The patient's neurological deficit fully recovered with immunosuppressants at the 3 month-follow-up. We report a rare case of a patient with TA initially presenting with AIS treated with bridging therapy with full recovery of neurological function. Bridging therapy should be taken into consideration for AIS in patients with TA. Further study is needed in this regard.
2013-05-17 | Takayasu's arteritis mimicking Kawasaki disease in 7-month-old infant, successfully treated with glucocorticosteroids and intravenous immunoglobulins.
Takayasu's arteritis (TA) rarely occurs in infants. There are only four reports on TA in children below 1 year of age, revealing diversity of its symptoms. We describe a 7-month-old female infant hospitalized for hectic fever, irritability, high acute phase indices, and coronary artery dilatations found on echocardiography, which suggested Kawasaki disease (KD). Despite of standard treatment, rapid development of thoracic and abdominal aorta aneurysms occurred, while coronary artery abnormalities regressed. The initial diagnosis was changed for TA. Subsequently implemented glucocorticosteroids led to clinical and laboratory normalization. However, 2 months after treatment discontinuation, TA relapsed. Glucocorticosteroid therapy was restarted with additional introduction of intravenous immunoglobulins (IVIG), resulting in full and stable remission lasting over 1 year. Thus, diagnosis and treatment of infant TA pose a big challenge to physicians. TA onset in infants may mimics KD. Prolonged glucocorticosteroids and IVIG administration can be recommended in the youngest patients with TA.
antibodies
2026-08-06 | Misty Mesentery as an Initial Imaging Finding Suggestive of Large Vessel Vasculitis.
Large vessel vasculitis (LVV) refers to vasculitis predominantly affecting large arteries, including the aorta and its major branches. It encompasses conditions such as giant cell arteritis and Takayasu arteritis, as well as unclassified or idiopathic forms. Imaging techniques, including computed tomography (CT), are essential for detecting large vessel involvement, particularly when biopsy findings are negative. We report the case of a 56-year-old man admitted with fever, headache, and upper abdominal pain lasting 2 weeks. On examination, he had scalp tenderness and upper abdominal tenderness without temporal artery enlargement. Laboratory tests revealed elevated C-reactive protein (CRP: 19.75 mg/dL) and normal IgG4 (45 mg/dL). Initial CT demonstrated increased attenuation of mesenteric fat (Figure 1), suggestive of misty mesentery. Antibiotic therapy for presumed infectious mesenteritis was ineffective, and CRP increased further to 30.20 mg/dL. Contrast-enhanced CT showed thickening of the aortic wall (Figure 2). Based on fever, headache, systemic inflammation, and aortic wall thickening, LVV was considered the most plausible diagnosis. Cite this article as: Noda S, Kondo S. Misty mesentery as an initial imaging finding suggestive of large vessel vasculitis. Eur J Rheumatol. 13(1), 0084, doi: 10.5152/eurjrheum.2026.25084.
2026-08-01 | Biological therapy in Takayasu arteritis. Results from the REVAS registry.
Biological therapy in patients with Takayasu arteritis is frequently used, but the factors associated with its use have not been determined. The present study aims to describe and identify predictors of the need for biological therapy in this vasculitis. We conducted a retrospective, multicentre, descriptive study including all patients with Takayasu arteritis enrolled in the REVAS registry from Spain. We performed a descriptive and a multivariate logistic regression analysis to identify variables associated with the need of biological therapy requirement. The study included 77 patients, 65 (84.4 %) women, with a median follow up of 9.9 (interquartile range 3.5-21.5) years. Sixty-three (81.8 %) participants received glucocorticoids and 45 (58.4 %) needed immunosuppressive therapy. Twenty-three patients had received biological therapy, 20 of whom were diagnosed after 2006. Biological therapy included infliximab (n = 3), adalimumab (n = 9) and tocilizumab (n = 18). Patients requiring biological therapy had a higher proportion of systemic symptoms (fever and/or constitutional syndrome) at baseline (70.0 %vs. 28.6 %, p = 0.005), received glucocorticoids more frequently (100.0 %vs. 78.6 %, p = 0.03) and required a greater number of immunosuppressants (2 (1-2) vs. 1 (0-1), p = 0.03) during follow-up. On multivariate logistic regression, the presence of systemic symptoms was associated with the need for biological therapy requirement (odds radio: 3.9 (1.02-14.8), p = 0.047). Conclusion: to our knowledge, this is the first study that evaluates variables associated with the need for biological therapy requirement in Takayasu arteritis. The presence of systemic symptoms at presentation was independently associated with the need for biological therapy, highlighting the importance of a thorough clinical evaluation.
2026-07-22 | Prognostic Value of the Hata Angiographic Classification for Long-Term Outcomes in Takayasu Arteritis: A Single-Center, Retrospective Study.
The prognostic utility of the Hata angiographic classification for long-term outcomes in Takayasu arteritis (TAK) remains unclear. To evaluate the prognostic value of the Hata classification for predicting major adverse cardiovascular events (MACE) and the need for vascular interventions in patients with TAK. This single-center, retrospective cohort study included patients diagnosed with TAK between 2000 and 2025. Multivariate logistic regression analyses were performed to identify independent predictors, with statistical significance defined as p < 0.05. Of 203 patients initially identified, 18 were excluded. The final cohort included 185 patients, of whom 80.0% were women, with a median age at diagnosis of 29 years. Type V was the predominant angiographic pattern (64.9%). During a median follow-up of 10.8 years, MACE occurred in 35.7% of patients, while 37.3% required vascular intervention. The Hata classification was not associated with MACE occurrence. Independent predictors of MACE included female sex, which was protective (odds ratio [OR], 0.38; 95% CI, 0.17-0.86; p = 0.021), and longer symptom onset-to-diagnosis time, which increased risk (OR, 1.10; 95% CI, 1.03-1.18; p = 0.006). In contrast, the Hata classification independently predicted the need for vascular intervention. Hata Type I showed a strong protective association compared with Type V (OR, 0.10; 95% CI, 0.01-0.53; p = 0.029). Baseline aortic insufficiency was also independently associated with a higher likelihood of intervention (OR, 2.54; 95% CI, 1.18-5.60; p = 0.018) (Central Illustration). The Hata classification appears to be a robust predictor of vascular intervention but not MACE in TAK. Greater anatomical disease extension (Type V) and baseline aortic insufficiency were associated with increased procedural risk, whereas MACE risk was associated with diagnostic delay and male sex. The observed long-term dissociation between high rates of clinical remission (92.4%) and continued disease progression underscores the importance of imaging-based surveillance and highlights the need for prospective multicenter studies to validate these findings.
2026-06-12 | Aortitis accelerates the growth rate of thoracic aortic aneurysms.
Thoracic aortic aneurysms (TAA) are predominantly degenerative. Non-infectious inflammatory aortitis, including giant cell arteritis (GCA), Takayasu arteritis and clinically isolated aortitis (CIA), represents a less understood aetiology with poorly characterised growth patterns. While GCA is linked to higher TAA risk, preoperative growth data for inflammatory TAA are scarce. This study compares growth rates in histologically proven inflammatory aortitis. We conducted a retrospective case-control study, including patients with histologically proven aortitis and matched controls with degenerative TAA. Cases and controls were matched for age, sex, surgical year and procedure type. Aneurysm growth rate (mm/month) was calculated from serial imaging. This study included 23 patients with histologically confirmed aortitis (13 GCA, 9 CIA) and 42 matched controls with degenerative TAA. The mean growth rate was significantly higher in the aortitis group (0.177±0.01 mm/month; 2.12 mm/year) compared with controls (0.039±0.02 mm/month; 0.47 mm/year), representing a 4.5-fold acceleration (p<0.001). Among inflammatory etiologies, CIA demonstrated faster growth (0.338±0.06 mm/month; 4.06 mm/year) than GCA (2.1 mm/year; p<0.01). Patients with CIA more frequently required Bentall procedures (33% vs 0%, p=0.047). No differences were observed in baseline demographics, cardiovascular risk factors or baseline aortic diameters between groups. Histologically, 70% of aortitis cases exhibited granulomatous inflammation. Inflammatory aortitis is associated with a 4.5-fold faster preoperative TAA growth rate compared with degenerative aneurysms, with CIA exhibiting two times the growth rate of GCA. Prospective studies are warranted to validate these results and optimise surveillance and management strategies for this high-risk population.
2026-06-06 | Incidental detection of Takayasu arteritis in a hypertensive pregnancy with recurrent pregnancy loss: a positive maternal outcome.
Takayasu arteritis, which is also known as aortic arch syndrome, non-specific aortoarteritis and pulseless disease, is a rare chronic inflammatory progressive large vessel vasculitis of likely autoimmune aetiology causing narrowing, occlusion and aneurysms of systemic and pulmonary arteries, especially the aorta and its branches, afflicting women of childbearing age. It is an uncommon disease with an estimated annual incidence rate of 1.2-2.6 per million. Hypertension is the the most common mode of presentation. Based on site and extent, it is divided into five types as type I (branches of aortic arch), type II (aortic arch, its branches and descending thoracic aorta), type III (descending thoracic aorta and abdominal aorta), type IV (abdominal aorta only) and type V (aortic arch, descending thoracic aorta and abdominal aorta). Therapeutic modalities include medical (antihypertensive, steroids, cyclophosphamide, etc) as well as surgical interventions.
other
2023-01-13 | [Analysis of differentially expressed microRNA and protein expression profiles carried by exosomes in the plasma of patients with Takayasu's arteritis].
Objective: To detect the microRNAs (miRNAs) and proteins carried by exosomes in the plasma of patients with newly diagnosed Takayasu's arteritis (TAK) and analyze their possible roles in the pathogenesis of TAK. Methods: Ten patients with newly diagnosed TAK from the Department of Rheumatology and Immunology, Peking Union Medical College Hospital were selected during June-November 2020. Five healthy controls were matched with five patients by age and sex. RNA sequencing and protein mass spectrometry were used to detect miRNAs and proteins, respectively, carried by exosomes in the plasma. Differentially expressed miRNAs (DE-miRNAs) and proteins (DEPs) were screened. Thereafter, hierarchical cluster analysis, function, signal pathway, and protein domain enrichment analysis of DE-miRNAs and DEPs were performed. Finally, miRNAs and proteins related to vasculitis and autoimmunity were identified. The possible roles of the miRNAs and proteins in the pathogenesis of TAK were explored. Enumeration data were compared using Fisher's exact probability test or Chi-square test, and a P-value<0.05 was considered significant. Results: Compared with the healthy controls, patients with TAK had 29 DE-miRNAs on their plasma exosomes. Among these DE-miRNAs, miR-101-3p, miR-122-5p, miR-143-3p, miR-185-3p, miR-192-5p, miR-194-5p, miR-19a-3p, miR-19b-3p, miR-20b-5p, miR-21-5p, miR-22-3p, miR-335-5p, miR-34a-5p, miR-3613-5p, miR-548ad-5p, miR-590-3p, and miR-7-5p were upregulated; whereas miR-1249-3p, miR-141-3p, miR-199a-5p, miR-199b-5p, miR-200a-3p, miR-200c-3p, miR-204-5p, miR-29c-5p, miR-335-3p, miR-381-3p, miR-4433b-5p, and miR-584-5p were downregulated. Finally, miR-34a-5p, miR-200c-3p, miR-143-3p, miR-22-3p, and miR-21-5p were identified. Among the 357 DEPs screened, 236 DEPs were upregulated, whereas 121 DEPs were downregulated. Finally, kallikrein B1 (KLKB1), kininogen 1 (KNG1), desmoplakin (DSP) were identified. Conclusion: MiR-34a-5p, miR-200c-3p, miR-143-3p, miR-22-3p, miR-21-5p, KLKB1, KNG1, and DSP carried by exosomes in plasma might participate in the pathogenesis of TAK by regulating vascular physiology, inflammation, autoimmunity, and other processes. They may be biomarkers and therapeutic targets of TAK. 目的： 检测初治多发性大动脉炎（TAK）患者血浆外泌体携带的微小RNA和蛋白，分析其在TAK发病机制中的可能作用。 方法： 选2020年6月至11月北京协和医院风湿免疫科确诊的初治TAK患者10例，另选与之年龄性别匹配的健康对照者5例，分别通过RNA测序和蛋白质谱技术检测血浆外泌体携带的微小RNA和蛋白，筛选出差异表达的微小RNA和蛋白，并对其进行层次聚类分析、功能、信号通路和结构域富集分析，最终筛选出与血管炎有关的微小RNA和蛋白，探究其在TAK发病机制中的可能作用。统计学采用Fisher精确概率检验或χ²检验。 结果： TAK患者血浆外泌体携带的差异表达的微小RNA 29个，其中miR-101-3p、miR-122-5p、miR-143-3p、miR-185-3p、miR-192-5p、miR-194-5p、miR-19a-3p、miR-19b-3p、miR-20b-5p、miR-21-5p、miR-22-3p、miR-335-5p、miR-34a-5p、miR-3613-5p、miR-548ad-5p、miR-590-3p、miR-7-5p表达上调，miR-1249-3p、miR-141-3p、miR-199a-5p、miR-199b-5p、miR-200a-3p、miR-200c-3p、miR-204-5p、miR-29c-5p、miR-335-3p、miR-381-3p、miR-4433b-5p、miR-584-5p表达下调。差异表达的蛋白357个，其中236个表达上调，121个表达下调，最终筛选前激肽释放酶、激肽原1、桥粒蛋白。 结论： 血浆外泌体携带的miR-34a-5p、miR-200c-3p、miR-143-3p、miR-22-3p、miR-21-5p、前激肽释放酶、激肽原1、桥粒蛋白可能通过调节血管生理过程、炎症反应、自身免疫等过程参与TAK的发病机制，具有作为TAK生物标志物和治疗靶点的潜在作用。.
Access all drug discovery papers and probability of success in trials forecasts:
Access all drug discovery papers and probability of success in trials forecasts:
Drug Discovery Landscape
2 orphan drug designations for Takayasu arteritis.
2 orphan drug designations for Takayasu arteritis.
Drug | Therapy type | Regulator | Orphan designation | Approval | Sponsor |
|---|---|---|---|---|---|
tocilizumab | antibodies | FDA | 2025-12-05 | — | ODDIFACT SAS |
adalimumab | antibodies | FDA | 2025-03-13 | — | ODDIFACT SAS |
Let's accelerate rare disease drug discovery
Let's accelerate drug discovery
Get access to Explority AI’s forecasts to outperform average preclinical success rates. Whether you’re expanding your R&D pipeline, evaluating a partnership, or simply have a question — we’d love to hear from you.