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Journal of Clinical Rheumatology :... Oct 2023To develop the first evidence-based Pan American League of Associations for Rheumatology (PANLAR) guidelines for the treatment of Takayasu arteritis (TAK).
OBJECTIVE
To develop the first evidence-based Pan American League of Associations for Rheumatology (PANLAR) guidelines for the treatment of Takayasu arteritis (TAK).
METHODS
A panel of vasculitis experts developed a series of clinically meaningful questions addressing the treatment of TAK patients in the PICO (population/intervention/comparator/outcome) format. A systematic literature review was performed by a team of methodologists. The evidence quality was assessed according to the GRADE (Grading of Recommendations/Assessment/Development/Evaluation) methodology. The panel of vasculitis experts voted each PICO question and made recommendations, which required ≥70% agreement among the voting members.
RESULTS
Eleven recommendations were developed. Oral glucocorticoids are conditionally recommended for newly diagnosed and relapsing TAK patients. The addition of nontargeted synthetic immunosuppressants (e.g., methotrexate, leflunomide, azathioprine, or mycophenolate mofetil) is recommended for patients with newly diagnosed or relapsing disease that is not organ- or life-threatening. For organ- or life-threatening disease, we conditionally recommend tumor necrosis factor inhibitors (e.g., infliximab or adalimumab) or tocilizumab with consideration for short courses of cyclophosphamide as an alternative in case of restricted access to biologics. For patients relapsing despite nontargeted synthetic immunosuppressants, we conditionally recommend to switch from one nontargeted synthetic immunosuppressant to another or to add tumor necrosis factor inhibitors or tocilizumab. We conditionally recommend low-dose aspirin for patients with involvement of cranial or coronary arteries to prevent ischemic complications. We strongly recommend performing surgical vascular interventions during periods of remission whenever possible.
CONCLUSION
The first PANLAR treatment guidelines for TAK provide evidence-based guidance for the treatment of TAK patients in Latin American countries.
Topics: Humans; United States; Takayasu Arteritis; Tumor Necrosis Factor Inhibitors; Rheumatology; Immunosuppressive Agents; Methotrexate
PubMed: 37553869
DOI: 10.1097/RHU.0000000000002004 -
Rheumatology (Oxford, England) Feb 2024To inform an international task force about current evidence on Treat to Target (T2T) strategies in PMR and GCA.
OBJECTIVES
To inform an international task force about current evidence on Treat to Target (T2T) strategies in PMR and GCA.
METHODS
A systematic literature research (SLR) was conducted in Medline, EMBASE, Cochrane Library, clinicaltrials.gov from their inception date to May 2022, and in the EULAR/ACR abstract database (2019-2021). Randomised clinical trials (RCTs) and non-randomised interventional studies published in English and answering at least one of the eleven PICO questions on T2T strategies, treatment targets and outcomes, framed by the taskforce, were identified. Study selection process, data extraction and risk of bias assessment were conducted independently by two investigators.
RESULTS
Of 7809 screened abstracts, 397 were selected for detailed review and 76 manuscripts were finally included (31 RCTs, eight subgroup/exploratory analyses of RCTs and 37 non-randomised interventional studies). No study comparing a T2T strategy against standard of care was identified. In PMR RCTs, the most frequently applied outcomes concerned treatment (90.9% of RCTs), particularly the cumulative glucocorticoids (GC) dose and GC tapering, followed by clinical, laboratory and safety outcomes (63.3% each). Conversely, the most commonly reported outcomes in RCTs in GCA were prevention of relapses (72.2%), remission as well as treatment-related and safety outcomes (67.0% each).
CONCLUSIONS
This SLR provides evidence and highlights the knowledge gaps on T2T strategies in PMR and GCA, informing the task force developing T2T recommendations for these diseases.
Topics: Humans; Giant Cell Arteritis; Polymyalgia Rheumatica; Glucocorticoids
PubMed: 37672017
DOI: 10.1093/rheumatology/kead471 -
Journal of Clinical Rheumatology :... Mar 2024Various studies have suggested interleukin 6 (IL-6) as a potential biomarker for detecting disease activity in Takayasu arteritis. (Meta-Analysis)
Meta-Analysis
BACKGROUND
Various studies have suggested interleukin 6 (IL-6) as a potential biomarker for detecting disease activity in Takayasu arteritis.
METHODS
A systematic review and meta-analysis was performed to assess differences in IL-6 levels in patients with active (aTA) and inactive Takayasu arteritis (iTA), as well as healthy controls (HCs), using validated activity scores. Study quality and the risk of bias were assessed using STROBE (Strengthening the Reporting of Observational Studies in Epidemiology) and the Newcastle-Ottawa and Joanna Briggs checklist, respectively. For the meta-analysis, we pooled the raw mean IL-6 levels in each group and then estimated and pooled the crude mean differences between the groups. We applied a random-effects model in all analyses.
RESULTS
Of the 93 eligible articles, 10 were included after removing duplicates and studies that met the exclusion criteria. Overall, 1825 patients with a mean age ranging from 24 to 40.6 years were included. The pooled levels of IL-6 increased depending on disease activity as follows: HCs: 3.08 (95% confidence interval [CI], 0.88-5.28), iTA: 7.21 (3.61-10.82), and aTA: 22.67 (12.44-32.91) pg/mL. Patients with aTA had higher IL-6 levels than HCs (21.52 [95% CI, -0.43 to 43.47]) and those with iTA (16.69 [95% CI, 5.32-28.06]), whereas IL-6 levels were not different between HCs and patients with iTA (3.62 [95% CI, -13.18 to 20.42]).
CONCLUSIONS
Interleukin 6 levels are significantly increased in patients with aTA compared with those with iTA and HCs but not in patients with iTA compared with HCs. More studies are needed to establish the IL-6 cutoff value for assessing disease activity.
Topics: Humans; Young Adult; Adult; Interleukin-6; Takayasu Arteritis; Biomarkers
PubMed: 38190729
DOI: 10.1097/RHU.0000000000002053 -
Arthritis Research & Therapy Jul 2023Cerebrovascular ischemic events (CIE) are among the most severe complications of giant cell arteritis (GCA). Heterogeneity between different studies in the definition of... (Meta-Analysis)
Meta-Analysis
BACKGROUND
Cerebrovascular ischemic events (CIE) are among the most severe complications of giant cell arteritis (GCA). Heterogeneity between different studies in the definition of GCA-related CIE leads to uncertainty regarding their real prevalence. The aim of our study was to evaluate the prevalence and describe the characteristics of GCA-related CIE in a well-phenotyped cohort completed by a meta-analysis of the existing literature.
METHODS
In this retrospective study performed in the Lille University Hospital, all consecutive patients with GCA according to American College of Rheumatology (ACR) diagnostic criteria were included from January 1, 2010, to December 31, 2020. A systematic review of the literature using MEDLINE and EMBASE was performed. Cohort studies of unselected GCA patients reporting CIE were included in the meta-analysis. We calculated the pooled summary estimate of GCA-related CIE prevalence.
RESULTS
A total of 271 GCA patients (89 males, mean age 72 ± 9 years) were included in the study. Among them, 14 (5.2%) presented with GCA-related CIE including 8 in the vertebrobasilar territory, 5 in the carotid territory, and 1 patient having multifocal ischemic and hemorrhagic strokes related to intra-cranial vasculitis. Fourteen studies were included in the meta-analysis, representing a total population of 3553 patients. The pooled prevalence of GCA-related CIE was 4% (95% CI 3-6, I = 68%). Lower body mass index (BMI), vertebral artery thrombosis on Doppler US (17% vs 0.8%, p = 0.012), vertebral arteries involvement (50% vs 3.4%, p < 0.001) and intracranial arteries involvement (50% vs 1.8%, p < 0.001) on computed tomography angiography (CTA) and/or magnetic resonance angiography (MRA), and axillary arteries involvement on positron emission computed tomography (PET/CT) (55% vs 20%, p = 0.016) were more frequent in GCA patients with CIE in our population.
CONCLUSIONS
The pooled prevalence of GCA-related CIE was 4%. Our cohort identified an association between GCA-related CIE, lower BMI, and vertebral, intracranial, and axillary arteries involvement on various imaging modalities.
Topics: Male; Humans; Middle Aged; Aged; Aged, 80 and over; Giant Cell Arteritis; Retrospective Studies; Positron Emission Tomography Computed Tomography; Carotid Arteries; Cohort Studies
PubMed: 37420252
DOI: 10.1186/s13075-023-03091-x -
International Journal of Infectious... Jan 2024Symptoms from SARS-CoV-2 infection can involve multiple organ systems. Several reviews discussed the neurologic involvement and neuroimaging findings in adults but...
OBJECTIVES
Symptoms from SARS-CoV-2 infection can involve multiple organ systems. Several reviews discussed the neurologic involvement and neuroimaging findings in adults but research on children is lacking. This study aimed to analyze the incidence of neurologic involvement in patients diagnosed with pediatric inflammatory multisystem syndrome temporally associated with COVID-19 (PIMS-TS) or multisystem inflammatory syndrome in children (MIS-C); and also to summarize current literature on possible neuroimaging findings in SARS-CoV-2 infected children.
METHODS
A literature search in six electronic databases was performed to retrieve case series, cohort studies, and cross-sectional studies on neurologic involvement in COVID-19 patients younger than 21 years of age published between December 2019 to September 2023, including COVID-19 patients.
RESULTS
A total of 2224 patients with MIS-C from 10 cohorts and cross-sectional studies suggested that neurologic involvement in these subsets ranges from 8.5% to 32.1%. Symptoms included acute encephalitis, seizures, stroke, cranial nerve palsy, nausea/vomiting, and intracranial hypertension. Neuroradiology findings of 114 children from 50 case reports included splenial or acute disseminated encephalomyelitis (ADEM)-like lesions, cytotoxic brain edema, autoimmune demyelinating diseases, ischemic stroke and arteritis, venous thrombosis, intracranial hemorrhage, meningitis, posterior reversible encephalopathy syndrome, anti-N-methyl-D-aspartate receptor autoimmune encephalitis, acute hemorrhagic leukoencephalitis, hydrocephalus, olfactory bulb atrophy, cerebellitis, and acute necrotizing encephalitis.
CONCLUSION
Radiologic findings of SARS-CoV-2 infection in the pediatric population are diverse. Neuroimaging studies should be considered in critically ill patients to rule out neurologic involvement and facilitate early interventions.
Topics: Adult; Humans; Child; COVID-19; SARS-CoV-2; Cross-Sectional Studies; Posterior Leukoencephalopathy Syndrome; Neuroimaging; Systemic Inflammatory Response Syndrome
PubMed: 37944584
DOI: 10.1016/j.ijid.2023.11.006 -
Seminars in Arthritis and Rheumatism Apr 2024A subset of Takayasu arteritis (TAK) has onset in the pediatric age group (≤18 years). The differences in mortality between pediatric-onset and adult-onset TAK are... (Meta-Analysis)
Meta-Analysis Review
Pediatric-onset Takayasu arteritis is associated with greater risk of mortality than adult-onset Takayasu arteritis-A systematic review with meta-analysis of observational cohort studies.
A subset of Takayasu arteritis (TAK) has onset in the pediatric age group (≤18 years). The differences in mortality between pediatric-onset and adult-onset TAK are unclear. Therefore, we undertook a systematic review with meta-analysis to compare mortality risk in pediatric-onset with adult-onset TAK. Scopus, Pubmed (MEDLINE and Pubmed Central), recent conference abstracts, clinicaltrials.gov, and the Cochrane database were searched up to August 2023 for relevant studies. Five studies (all of moderate or high quality on the Newcastle Ottawa scale) were identified which had compared mortality between 151 pediatric-onset and 499 adult-onset TAK. Pediatric-onset TAK was associated with a significantly higher risk of death than adult-onset TAK (pooled risk ratio 2.27, 95% confidence interval 1.05 - 4.85, I=0%). Cardiovascular disease and infections were the major causes of death in both pediatric-onset and adult-onset TAK. Sub-group analyses identified a greater mortality risk with pediatric-onset TAK in retrospective (but not prospective) studies and in studies of high quality (but not in those of moderate quality). Meta-regression did not reveal a significant influence of differences in sex distribution or age or the proportions of patients with pediatric-onset or adult-onset TAK on the pooled mortality risk. An increased mortality risk with pediatric-onset TAK on meta-analysis is consistent with more frequent severe organ manifestations of pediatric-onset TAK (heart failure, renal failure) when compared with adult-onset TAK. Future studies should systematically evaluate differences in the pathogenesis between pediatric-onset and adult-onset to understand the reasons for such observed differences in the mortality risk.
Topics: Adult; Humans; Child; Adolescent; Retrospective Studies; Takayasu Arteritis; Cohort Studies; Cardiovascular Diseases; Prospective Studies; Observational Studies as Topic
PubMed: 38183753
DOI: 10.1016/j.semarthrit.2023.152355 -
Rheumatology International Jul 2024VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome is a newly discovered autoinflammatory condition characterised by somatic mutation of the UBA1... (Review)
Review Meta-Analysis
BACKGROUND
VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome is a newly discovered autoinflammatory condition characterised by somatic mutation of the UBA1 gene. The syndrome leads to multi-system inflammation affecting predominantly the skin, lungs and bone marrow.
METHODS
We undertook a systematic review of the multisystem features and genotypes observed in VEXAS syndrome. Articles discussing VEXAS syndrome were included. Medline, Embase and Cochrane databases were searched. Information was extracted on: demographics, type and prevalence of clinical manifestations, genetic mutations and treatment. Meta-analysis using a random effects model was used to determine pooled estimates of serum markers.
RESULTS
From 303 articles, 90 were included, comprising 394 patients with VEXAS. 99.2% were male, with a mean age of 67.1 years (SD 8.5) at disease onset. The most frequent diagnoses made prior to VEXAS were: relapsing polychondritis (n = 59); Sweet's syndrome (n = 24); polyarteritis nodosa (n = 11); and myelodysplastic syndrome (n = 10). Fever was reported in 270 cases (68.5%) and weight loss in 79 (20.1%). Most patients had haematological (n = 342; 86.8%), dermatological (n = 321; 81.5%), pulmonary (n = 297; 75.4%%) and musculoskeletal (n = 172; 43.7%) involvement, although other organ manifestations of varying prevalence were also recorded. The most commonly reported mutations were "c.122T > C pMET41Thr" (n = 124), "c.121A > G pMET41Val" (n = 62) and "c.121A > C pMet41Leu" (n = 52). Most patients received glucocorticoids (n = 240; 60.9%) followed by methotrexate (n = 82; 20.8%) and IL-6 inhibitors (n = 61, 15.4%). One patient underwent splenectomy; 24 received bone marrow transplants.
CONCLUSION
VEXAS syndrome is a rare disorder affecting predominantly middle-aged men. This is the first systematic review to capture clinical manifestations, genetics and treatment of reported cases. Further studies are needed to optimise treatment and subsequently reduce morbidity and mortality.
Topics: Humans; Male; Ubiquitin-Activating Enzymes; Female; Mutation; Syndrome; Aged; Middle Aged; Myelodysplastic Syndromes; Sweet Syndrome; Polyarteritis Nodosa; Hereditary Autoinflammatory Diseases
PubMed: 38129348
DOI: 10.1007/s00296-023-05513-0 -
Zeitschrift Fur Rheumatologie Nov 2023To update the estimated prevalence of inflammatory rheumatic diseases (IRD) in Germany.
OBJECTIVE
To update the estimated prevalence of inflammatory rheumatic diseases (IRD) in Germany.
METHODS
A systematic literature search in PubMed and Web of Science (last search 8 November 2022) identified original articles (regional and nationwide surveys and routine data analyses for arthritides, connective tissue diseases, and vasculitides) on the prevalence for the period 2014-2022. Data sources, collection period, case definition, and risk of bias are reported. The prevalences were estimated from available national data, with consideration of international data.
RESULTS
Screening by 2 authors yielded 263 hits, of which 18 routine data analyses and 2 surveys met the inclusion criteria. Prevalence data ranged from 0.42% to 1.85% (rheumatoid arthritis), 0.32-0.5% (ankylosing spondylitis), 0.11-0.32% (psoriatic arthritis), 0.037-0.14% (systemic lupus erythematosus), 0.07-0.77% (Sjoegren's disease/sicca syndrome), 0.14-0.15% (polymyalgia rheumatica, ≥ 40 years), 0.04-0.05% (giant cell arteritis, ≥ 50 years), and 0.015-0.026% (ANCA-associated vasculitis). The risk of bias was moderate in 13 and high in 7 studies. Based on the results, we estimate the prevalence of IRD in Germany to be 2.2-3.0%, which corresponds to approximately 1.5-2.1 million affected individuals. Prevalence data of juvenile idiopathic arthritis was reported to be around 0.10% (0.07-0.10%) of patients 0-18 years old, corresponding to about 14,000 children and adolescents in Germany.
CONCLUSION
This systematic review shows an increase in the prevalence of IRD in Germany, which is almost exclusively based on routine data analyses. In the absence of multistage population studies, the available data are overall uncertain sources for prevalence estimates at moderate to high risk of bias.
Topics: Child; Adolescent; Humans; Infant, Newborn; Infant; Child, Preschool; Prevalence; Arthritis, Rheumatoid; Spondylitis, Ankylosing; Arthritis, Juvenile; Sjogren's Syndrome; Rheumatic Fever; Giant Cell Arteritis; Rheumatic Diseases
PubMed: 36592211
DOI: 10.1007/s00393-022-01305-2 -
Acta Neurologica Belgica Oct 2023
PubMed: 34491519
DOI: 10.1007/s13760-021-01763-6 -
Revista Clinica Espanola Jun 2024Systematic review of current evidence to analyze the prevalence of extracranial large vessel vasculitis (LVV) using F-FDG PET/CT in patients with polymyalgia rheumatica...
OBJECTIVE
Systematic review of current evidence to analyze the prevalence of extracranial large vessel vasculitis (LVV) using F-FDG PET/CT in patients with polymyalgia rheumatica (PMR) or giant cell arteritis (GCA).
MATERIALS AND METHODS
PubMed and EMBASE were searched and the results were screened by two reviewers. Study quality was assessed using a modified version of the Newcastle-Ottawa scale. Heterogeneity between studies was assessed using the I statistic and the Q test. Further subgroup analyses were performed by disease type, study quality, and F-FDG PET/CT uptake criteria. Publication bias was assessed by funnel plot and Egger's test.
RESULTS
268 publications were identified, of which 17 met the selection criteria and were included in the meta-analysis. The overall pooled prevalence of extracranial LVV by F-FDG PET/CT was 54.5% [95% CI: 42.6%-66.1%]. In patients with GCA the prevalence was significantly higher than in patients with PMR (60.1% vs. 41.8%, P = 0.006). Likewise, studies with a lower risk of bias reported a higher prevalence of extracranial LVV (61.1% vs. 46.9%; P = 0.010). No publication bias was observed.
CONCLUSIONS
The F-FDG PET/CT test may be useful in the detection of extracranial LVV, both in patients with PMR or GCA. Such involvement is more frequent in patients with GCA, and may vary depending on the quality of the studies.
PubMed: 38852739
DOI: 10.1016/j.rceng.2024.06.005