Para médicos · reumatologia
Resumos de artigos, podcasts e newsletters sobre Arterite de Takayasu, para atualização médica.
A newsletter destaca a revisão do índice de dano (SDI) no Lúpus Eritematoso Sistêmico, que busca maior precisão clínica ao remover itens de atividade inflamatória e incluir critérios de gravidade. Além disso, apresenta novos guidelines da EULAR para vasculites e polimialgia reumática, e discute evidências recentes sobre terapias biológicas e inibidores de fosfodiesterase em doenças autoimunes.
Takayasu arteritis (TAK) is a large-vessel vasculitis in which glomerulonephritis is a rare complication with a poorly characterized clinical and histopathological profile. Patients with biopsy-proven glomerulonephritis were retrospectively identified from a single-center TAK cohort followed between 2014 and 2022. A systematic review of PubMed/MEDLINE, Scopus, and Web of Science for articles published up to December 2025 was conducted in parallel, excluding studies involving patients younger than 18 years and cases with confounding autoimmune conditions or non-classifiable biopsy findings. This case-based review was reported in accordance with the CABARET and PRISMA 2020 standards. Among 72 patients with TAK, three (4.2%) had biopsy-proven glomerulonephritis. The systematic review yielded 31 articles describing 39 additional cases (84.6% female; median age at TAK diagnosis 26 years; median age at glomerular disease diagnosis 33 years). Glomerular disease was diagnosed a median of 5 years (IQR 0-11) after TAK and most often presented as asymptomatic proteinuria or nephrotic syndrome. Mesangial proliferative glomerulonephritis was the most common subtype (35.9%), followed by AA amyloidosis (30.8%), membranoproliferative glomerulonephritis (12.8%), focal segmental glomerulosclerosis (10.3%), and membranous nephropathy (7.7%). Renal artery stenosis was absent in 69.2% of cases. Corticosteroids were used in 92.3% of cases and biologic agents in 10.3%. Glomerulonephritis is a rare but clinically significant late complication of TAK, characterized by a distinct histopathological spectrum dominated by mesangial lesions, AA amyloidosis, and membranoproliferative glomerulonephritis. Routine proteinuria screening in patients with TAK, including during clinical remission, is warranted to enable early detection and timely management.
A newsletter aborda a importância da reabilitação imunológica pós-transplante renal em pacientes com doenças reumáticas, focando no equilíbrio entre evitar a rejeição e prevenir a recorrência da doença de base. Destaca o sucesso do brepocitinibe (inibidor de TYK2/JAK1) no tratamento da dermatomiosite refratária e discute o uso de terapias biológicas combinadas para artrite psoríasica. O conteúdo também revisa biomarcadores preditivos e estratégias de monitoramento personalizado para otimizar a sobrevida do enxerto e do paciente.
Takayasu arteritis (TA) is a chronic, immune-mediated vasculitis that primarily affects the large arteries, particularly the aorta and its main branches, leading to stenosis, occlusion, dilation, or aneurysms. Traditional imaging modalities, including computed tomography angiography (CTA), magnetic resonance angiography (MRA), and positron emission tomography/computed tomography (PET-CT), offer valuable diagnostic information; however, their clinical applicability has limitations, including high costs, radiation exposure, and reduced availability. In recent years, contrast-enhanced ultrasound (CEUS) has emerged as a minimally invasive, radiation-free technique capable of detecting vascular wall neovascularization as a potential surrogate imaging marker of TA inflammatory activity. This scoping review, conducted in accordance with the Joanna Briggs Institute (JBI) methodology and the Preferred Reporting Items for Systematic Reviews and Meta-Analyses extension for Scoping Reviews (PRISMA-ScR) checklist, aims to synthesize current evidence on the diagnostic and monitoring utility of CEUS in patients with TA and carotid involvement. We systematically searched MEDLINE/PubMed, Scopus, Web of Science, LILACS and EBSCO from inception to May 2025 for original studies of adult patients with TA in whom CEUS was used to assess disease activity. Eighteen studies (8 cross-sectional, 3 cohort, 7 case reports; 631 patients) met the eligibility criteria. Across these studies, CEUS detected arterial wall neovascularization compatible with active inflammation, particularly in the carotid arteries, and demonstrated moderate-to-strong correlations with inflammatory biomarkers, PET-CT findings, and clinical activity scores such as the Indian Takayasu Activity Score (ITAS) and the National Institutes of Health (NIH) criteria. Importantly, CEUS can reveal subclinical or residual inflammation even when clinical symptoms or laboratory markers are absent. Its ability to detect early relapses and monitor therapeutic response has been demonstrated in both observational cohorts and individual case reports. Despite this, substantial heterogeneity in
Abstract Stroke represents a major complication in Takayasu arteritis (TA). We aimed to determine clinical characteristics and neurological outcomes in TA patients with stroke compared to those without. We retrospectively analyzed 35 patients (27F/8 M) with documented stroke to 50 consecutive patients (47F/3 M) without stroke followed by the Istanbul University-Cerrahpasa Medical Faculty. Demographic data, clinical manifestations, arterial involvement patterns, treatments, and neurological outcomes were evaluated. Disability was assessed using the Expanded Disability Status Scale (EDSS), Barthel Index, and Modified Rankin Scale. Mean age at diagnosis among patients with stroke and non-stroke was similar (38.5 ± 10.7 vs. 35.6 ± 11.6 years). The mean age at stroke was 43.1 ± 10.3 years. Patients with stroke were more likely to be male (22.9% vs. 6.0%, p = 0.023). Strokes were predominantly ischemic (91.4%), affecting anterior circulation (82.8%) with left hemisphere predominance (72.4%). Internal carotid artery (ICA) involvement was significantly associated with stroke (right ICA: 51.4% vs 18.0%, p = 0.001; left ICA: 37.1% vs 18.0%, p = 0.047), while abdominal aorta involvement seemed to be protective (20.0% vs 42.0%, p = 0.028). Male gender (OR = 5.70, p = 0.038) and any ICA involvement (OR = 5.98, p = 0.004) were identified as independent predictors of stroke. Importantly, 40% experienced stroke as the initial TA manifestation. Among those developing stroke after TA diagnosis, 85.7% were already receiving immunosuppression and 47.6% antiplatelet therapy. Stroke patients demonstrated significant disability (mean EDSS: 3.63 ± 3.36 vs 0.02 ± 0.14, p < 0.001) and 11.4% mortality, median 5 years after stroke. Male patients and those with ICA involvement face the highest risk for stroke in TA. Long-term consequences are devastating with increased mortality, severe disability
Aortitis associated with giant cell arteritis (GCA) is a severe manifestation, potentially leading to aneurysms and aortic dissection. Tocilizumab (TCZ) has demonstrated efficacy in the treatment of GCA, both intravenously or subcutaneously administered. However, pivotal studies did not specifically evaluate aortic involvement, and no comparison of intravenous (IV) versus subcutaneous (SC) TCZ has been performed in patients with GCA-related aortitis. The objective of this study was to compare the effectiveness of TCZ according to the administration route in patients with GCA-associated aortitis under clinical practice conditions. This was a multicenter observational study including 196 patients diagnosed with GCA-associated aortitis by imaging and treated with TCZ. Patients were grouped by administration route: IV or SC. GCA was diagnosed following the 1990 American College of Rheumatology criteria, temporal artery biopsy, and/or vascular imaging. Aortitis was identified using 18F-fluorodeoxyglucose positron emission tomography/computed tomography scan. Main outcomes included EULAR remission, clinical and imaging remission, absence of systemic inflammation, and glucocorticoid-sparing effect. Of 196 patients (148 women; mean age 69.8 ± SD 9.4 years), 110 received IV TCZ and 86 SC TCZ. Baseline clinical characteristics and markers of inflammation were comparable between groups. The glucocorticoid-sparing effect was similar. At 24-month follow-up, EULAR-defined remission was significantly more frequent in the SC group (83.3% vs 80.6%; P < 0.05). However, rates of imaging remission and absence of systemic inflammation were comparable between treatment arms. In this real-world cohort of GCA-associated aortitis, SC TCZ showed slightly greater effectiveness than IV TCZ in achieving EULAR-defined remission, whereas no significant differences were observed between both routes regarding imaging remission.
Rheumatic disease registries systematically collect real-world longitudinal data, improving patient care and research. Despite the high burden of rheumatic diseases, national registries are scarce in the Middle East. Rheumatology Research Center, with support from Iran’s Ministry of Health, launched Rheumatry in 2016 as the country’s first national rheumatic disease registry. To outline the design, governance, and data collection framework of Rheumatry. Rheumatry is a secure, web-based, multicenter registry led by Tehran University’s Rheumatology Research Center. It enrolls patients meeting standardized criteria for major rheumatic diseases (rheumatoid arthritis, Systemic lupus erythematosus, systemic sclerosis, ankylosing spondylitis, Takayasu arteritis, etc.) from over 26 centers nationwide. Comprehensive electronic case report forms (intake and follow-up) capture demographics, disease history, clinical features, laboratory and imaging results, standardized disease activity measures, patient-reported outcomes, and treatment data. As of September 2025, Rheumatry includes ~ 11,900 patients and 26,100 visits. Its multi-disease scope, inclusion of Takayasu arteritis, and alignment with international standards (ACR/EULAR criteria, MedDRA coding) are notable innovations. The registry supports systematic monitoring of disease courses, therapies, and outcomes, addressing a regional gap in real-world evidence. Rheumatry’s national-scale infrastructure provides detailed longitudinal data on diverse rheumatic diseases in Iran. By facilitating research, surveillance, and quality improvement, it bridges a critical information gap in the Middle Eastern context.
Stroke presentation differs between GCA and TAK, with predominant vertebrobasilar involvement in GCA. Carotid involvement is more frequent in TAK and often requires vascular procedures. These differences are crucial for appropriate management.
This study included patients diagnosed with Takayasu's arteritis (TAK) according to the 2022 American College of Rheumatology/European Alliance of Associations for Rheumatology classification criteria for TAK and compared the frequencies of items satisfied in the 2022 criteria between patients with young-onset TAK (YOTAK) and those with lateonset TAK (LOTAK). The medical records of 138 patients with TAK were retrospectively reviewed. YOTAK was arbitrarily defined as TAK diagnosed at 20-40 years of age, whereas LOTAK was defined as TAK classified at 41-60 years of age. The analyses were conducted by assessing and comparing the frequencies of items that fulfilled the 2022 criteria for TAK. The median age of the 138 patients diagnosed with TAK was 45.0 years, and 89.1% of the patients were female. Of the 138 patients, 47 and 91 were allocated to the YOTAK and LOTAK groups. Patients with LOTAK exhibited significantly higher frequencies of the items of vascular bruit (85.7% vs. 70.2%, OR 2.55 (95% CI 1.08-6.00), p=0.030), reduced pulse in upper extremities (73.6% vs. 42.6%, OR 3.77 (95% CI 1.79-7.92), p<0.001), and systolic blood pressure difference in arms (96.7% vs. 87.2%, OR 4.29 (95% CI 1.02-18.02), p=0.033) than those with YOTAK. Conversely, the involvement of the ascending aorta was significantly more frequently found in patients with YOTAK than those with LOTAK (19.1% vs. 7.7%, OR 0.35 (95% CI 0.12-0.99), p=0.046). Results of this study revealed that patients with LOTAK exhibited higher frequencies of vascular bruit, reduced brachial arterial pulse, and systolic blood pressure differences in arms, but a lower frequency of ascending aorta involvement than those with YOTAK.
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.
To compare diffusion-weighted whole-body imaging with background body signal suppression (DWIBS) with ^18F-fluorodeoxyglucose positron emission tomography/computed tomography (FDG-PET/CT) for assessing disease activity in large-vessel vasculitis (LVV), and to evaluate whether segment-level analysis provides complementary information beyond global grading. We enrolled 25 patients with LVV (Takayasu arteritis [TAK], n = 11; giant cell arteritis [GCA], n = 14). Two blinded readers graded 11 arterial territories (18 segments) on a four-point visual scale for DWIBS and FDG-PET/CT. Global imaging grades were calculated as segment-averaged means. Correlations with serum C-reactive protein (CRP) and erythrocyte sedimentation rate (ESR) were assessed. Global mean DWIBS grades correlated significantly with FDG-PET/CT grades (r = 0.72) and with CRP levels (r = 0.53). Territory-level mapping revealed diffuse central aortic involvement in GCA, whereas TAK showed more focal and segment-limited patterns. DWIBS demonstrated lower sensitivity in motion-prone regions but appeared to better reflect clinical activity and more frequently detected subclavian artery involvement in TAK compared with FDG-PET/CT. Residual vascular signals were also observed in some patients classified as being in clinical remission. DWIBS shows good agreement with FDG-PET/CT and provides a radiation- and contrast-free approach for assessing LVV. Global mean grades alone showed limited discriminatory power and may underestimate disease activity in focal or segment-limited involvement. Integrating segment-level imaging findings with clinical and laboratory markers may improve assessment of disease activity. Multicenter validation and longitudinal studies are warranted.
To explore the clinical and disease characteristics associated with aortic ulcers signs on computed tomography angiography (CTA) in patients with Takayasu's arteritis (TAK). We retrospectively analysed CTA scans from consecutive TAK patients at Xijing Hospital between 2021 and 2025. We identified aortic ulcers and recorded their location, transverse diameter, and depth. Clinical, laboratory, and imaging data were compared between ulcer and non-ulcer groups. Multivariate regression analysed independent risk factors for ulcers. The prevalence of aortic ulcers was 7.6% (27/355) among overall screened TAK patients. Of 200 hospitalised patients in this study, 26 (13.0%) exhibited signs of aortic ulcers. Ulcers were predominantly located in the thoracic aorta, aortic arch, and left common carotid artery, with a median transverse diameter of 3.4 mm and a depth of 2.4 mm. The ulcer group demonstrated significantly higher rates of Numano V (69.2% vs. 40.8%, p=0.007), aortic regurgitation (52.4% vs. 21.8%, p=0.003), and T-SPOT TB positivity (43.5% vs. 19.2%, p=0.01). No significant differences in disease activity scores or most inflammatory markers were observed. Numano V was identified as an independent risk factor for the presence of aortic ulcers (OR 3.45, 95% CI 1.38-8.61, p=0.008). Follow-up CTAs in 5 patients indicated stable ulcer size despite vascular progression. In TAK patients, aortic ulcers were independently associated with Numano V, but not with current systemic activity, suggesting they may represent chronic structural sequelae. Long-term monitoring is essential to reduce the risks of complications.