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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 -
Joint Bone Spine Jul 2023Giant Cell Arteritis (GCA) is a heterogenous systemic granulomatous vasculitis involving the aorta and any of its major tributaries. Despite increased awareness of large... (Meta-Analysis)
Meta-Analysis
BACKGROUND
Giant Cell Arteritis (GCA) is a heterogenous systemic granulomatous vasculitis involving the aorta and any of its major tributaries. Despite increased awareness of large vessel (LV) involvement, studies reporting incidence, clinical characteristics and complications of large-vessel GCA (LV-GCA) show conflicting results due to inconsistent disease definitions, differences in study methodologies and the broad spectrum of clinical presentations. The aim of this systematic literature review was to better define LV-GCA based on the available literature and identify distinguishing characteristics that may differentiate LV-GCA patients from those with limited cranial disease.
METHODS
Published studies indexed in MEDLINE and EMBASE were searched from database inception to 7th May 2021. Studies were included if they presented cohort or cross-sectional data on a minimum of 25 patients with LV-GCA. Control groups were included if data was available on patients with limited cranial GCA (C-GCA). Data was quantitatively synthesised with application of a random effects meta-regression model, using Stata.
RESULTS
The search yielded 3488 studies, of which 46 were included. Diagnostic criteria for LV-GCA differed between papers, but was typically dependent on imaging or histopathology. Patients with LV-GCA were generally younger at diagnosis compared to C-GCA patients (mean age difference -4.53 years), had longer delay to diagnosis (mean difference 3.03 months) and lower rates of positive temporal artery biopsy (OR: 0.52 [95% CI: 0.3, 0.91]). Fewer LV-GCA patients presented with cranial manifestations and only 53% met the 1990 ACR Classification Criteria for GCA. Vasculitis was detected most commonly in the thoracic aorta, followed by the subclavian, brachiocephalic trunk and axillary arteries. The mean cumulative prednisolone dose at 12-months was 6056.5mg for LV-GCA patients, relapse rates were similar between LV- and C-GCA patients, and 12% of deaths in LV-GCA patients could be directly attributed to an LV complication.
CONCLUSION
Patients with LV-GCA have distinct disease features when compared to C-GCA, and this has implications on diagnosis, treatment strategies and surveillance of long-term sequalae.
Topics: Humans; Giant Cell Arteritis; Cross-Sectional Studies; Phenotype
PubMed: 36858169
DOI: 10.1016/j.jbspin.2023.105558 -
Transplant Infectious Disease : An... Dec 2022The role of culturing the graft preservation fluid (PF) is controversial and its impact on graft arteritis development remains unclear. (Meta-Analysis)
Meta-Analysis
BACKGROUND
The role of culturing the graft preservation fluid (PF) is controversial and its impact on graft arteritis development remains unclear.
METHODS
Systematic literature search retrieving observational studies comparing solid organ transplant (SOT) recipients with culture-positive PF versus culture-negative PF. The quality of included studies was independently assessed according to the ROBINS-I tool for observational studies. Meta-analysis was performed using Mantel-Haenszel random-effect models. Graft site arteritis within 180 days from transplant was selected as the primary outcome.
RESULTS
Twenty-one observational studies (N = 2208 positive PF vs. 4458 negative) were included. Among positive PF, 857 (38.8%) were classified as high-risk group pathogens and 1351 (61.2%) as low-risk pathogens. Low-risk and negative PF showed similar odds ratios. A significant higher risk of graft arteritis was found in SOT recipients with a PF yielding a high-risk pathogen (odds ratio [OR] 18.43, 95% confidence interval [CI] 7.83-43.40) compared to low-risk and negative PF, with low heterogeneity (I = 2.24%). Similar results were found considering separately high-risk bacteria (OR 12.02, 95%CI 4.88-29.60) and fungi (OR 71.00, 95%CI 28.07-179.56), with no heterogeneity (I = 0%), and in the subgroup analyses of the liver (OR 16.78, 95%CI 2.95-95.47) and kidney (OR 19.90, 95%CI 4.78-82.79) recipients. However, data about diagnostic features of graft arteritis were very limited, indeed for only 11 of the 93 events histological or microbiological results were reported.
CONCLUSIONS
Our results may support the performance of PF culturing and a preemptive diagnostic or therapeutic management upon isolation of high-risk pathogens. Further studies based on a reliable diagnosis of graft arteritis are needed.
Topics: Humans; Liver; Fungi; Bacteria; Arteritis
PubMed: 36271646
DOI: 10.1111/tid.13979 -
Journal of Clinical Rheumatology :... Sep 2023The aim of this meta-analysis was to estimate the mean duration of glucocorticoid (GC) treatment in patients with giant cell arteritis. PubMed, EMBASE, and Cochrane... (Meta-Analysis)
Meta-Analysis
The aim of this meta-analysis was to estimate the mean duration of glucocorticoid (GC) treatment in patients with giant cell arteritis. PubMed, EMBASE, and Cochrane databases were searched from inception until November 30, 2021. The outcome measures were the proportion of patients on GCs at years 1, 2, and 5 after diagnosis and the mean GC dose (in the entire cohort and expressed in prednisone equivalents) at these time points. Twenty-two studies involving a total of 1786 patients were included. The pooled proportions of patients taking GCs at years 1, 2, and 5 were 89.7% (95% confidence interval [CI], 83.2%-93.9%), 75.2% (95% CI, 58.7%-86.6%), and 44.3% (95% CI, 15.2%-77.6%), respectively. The pooled GC dose at years 1 and 2 was 9.1 mg/d (95% CI, 2.8-15.5 mg/d) and 7.8 mg/d (95% CI, 1.4-14.1 mg/d), respectively. The proportion of patients taking GCs at year 1 was lower in multicenter studies ( p = 0.003), in randomized controlled trials ( p = 0.01), and in studies using a GC-tapering schedule ( p = 0.01). There were no significant differences in the proportion of patients taking GCs at years 1 and 2 according to study design (retrospective vs. prospective), initial GC dose, use of pulse GCs, publication year, enrolment period, duration of follow-up, age, and sex. This meta-analysis showed that giant cell arteritis is a chronic disease that requires substantial and prolonged GC treatment in a considerable proportion of patients. A predefined GC-tapering schedule may help to avoid inadequately long GC treatment.
Topics: Humans; Duration of Therapy; Glucocorticoids; Giant Cell Arteritis; Chronic Disease; Treatment Outcome
PubMed: 36126266
DOI: 10.1097/RHU.0000000000001897 -
Orphanet Journal of Rare Diseases Oct 2020Rare diseases (RDs) in rheumatology as a group have a high prevalence, but randomized controlled trials are hampered by their heterogeneity and low individual... (Meta-Analysis)
Meta-Analysis Review
BACKGROUND
Rare diseases (RDs) in rheumatology as a group have a high prevalence, but randomized controlled trials are hampered by their heterogeneity and low individual prevalence. To survey the current evidence of pharmacotherapies for rare rheumatic diseases, we conducted a systematic review and meta-analysis. Randomized controlled trials (RCTs) of RDs in rheumatology for different pharmaco-interventions were included into this meta-analysis if there were two or more trials investigating the same RD and using the same assessment tools or outcome parameters. The Cochrane Central Register of Controlled Trials (CENTRAL), MEDLINE, Embase, and PUBMED were searched up to April 2nd 2020. The overall objective of this study was to identify RCTs of RDs in rheumatology, evaluate the overall quality of these studies, outline the evidence of pharmacotherapy, and summarize recommended therapeutic regimens.
RESULTS
We screened 187 publications, and 50 RCTs met our inclusion criteria. In total, we analyzed data of 13 different RDs. We identified several sources of potential bias, such as a lack of description of blinding methods and allocation concealment, as well as small size of the study population. Meta-analysis was possible for 26 studies covering six RDs: Hunter disease, Behçet's disease, giant cell arteritis, ANCA-associated vasculitis, reactive arthritis, and systemic sclerosis. The pharmacotherapies tested in these studies consisted of immunosuppressants, such as corticosteroids, methotrexate and azathioprine, or biologicals. We found solid evidence for idursulfase as a treatment for Hunter syndrome. In Behçet's disease, apremilast and IF-α showed promising results with regard to total and partial remission, and Tocilizumab with regard to relapse-free remission in giant cell arteritis. Rituximab, cyclophosphamide, and azathioprine were equally effective in ANCA-associated vasculitis, while mepolizumab improved the efficacy of glucocorticoids. The combination of rifampicin and azithromycin showed promising results in reactive arthritis, while there was no convincing evidence for the efficacy of pharmacotherapy in systemic sclerosis.
CONCLUSION
For some diseases such as systemic sclerosis, ANCA-associated vasculitis, or Behcet's disease, higher quality trials were available. These RCTs showed satisfactory efficacies for immunosuppressants or biological drugs, except for systemic sclerosis. More high quality RCTs are urgently warranted for a wide spectrum of RDs in rheumatology.
Topics: Humans; Immunosuppressive Agents; Methotrexate; Rare Diseases; Rheumatic Diseases; Rituximab
PubMed: 33129321
DOI: 10.1186/s13023-020-01576-5 -
Seminars in Arthritis and Rheumatism Dec 2014To investigate the association between giant cell arteritis (GCA)/polymyalgia rheumatica (PMR) and malignancy risk. (Meta-Analysis)
Meta-Analysis Review
OBJECTIVE
To investigate the association between giant cell arteritis (GCA)/polymyalgia rheumatica (PMR) and malignancy risk.
METHODS
We conducted a systematic review and meta-analysis of cohort studies that reported relative risk, hazard ratio, or standardized incidence ratio (SIRs) with 95% confidence comparing malignancy risk in patients with GCA/PMR versus non-GCA/PMR participants. Pooled risk ratios and 95% confidence intervals were calculated using a random-effect, generic inverse variance method.
RESULT
A total of six studies were identified and included in our data analysis. The pooled risk ratio of malignancy in patients with GCA/PMR was 1.14 (95% CI: 1.05-1.22). The risk was higher in the first 6-12 months after diagnosis with the pooled risk ratio of 2.16 (95% CI: 1.85-2.53). However, when we performed a sensitivity analysis that excluded one study with a potential selection bias, the pooled risk ratio decreased and did not achieve statistical significance.
CONCLUSION
Our study demonstrated a low but statistically significant increased malignancy risk among patients with GCA/PMR. However, when we excluded one study with potential selection bias, the new pooled risk ratio did not achieve statistical significance.
Topics: Giant Cell Arteritis; Humans; Incidence; Neoplasms; Polymyalgia Rheumatica; Risk Factors
PubMed: 25074657
DOI: 10.1016/j.semarthrit.2014.06.004 -
Clinical Rheumatology Nov 2021The pharmacotherapy of Takayasu arteritis (TAK) with disease-modifying anti-rheumatic drugs (DMARDs) is an evolving area. A systematic review of Scopus, Web of Science,... (Meta-Analysis)
Meta-Analysis Review
The pharmacotherapy of Takayasu arteritis (TAK) with disease-modifying anti-rheumatic drugs (DMARDs) is an evolving area. A systematic review of Scopus, Web of Science, Pubmed Central, clinical trial databases and recent international rheumatology conferences for interventional and observational studies reporting the effectiveness of DMARDs in TAK identified four randomized controlled trials (RCTs, with another longer-term follow-up of one RCT) and 63 observational studies. The identified trials had some concern or high risk of bias. Most observational studies were downgraded on the Newcastle-Ottawa scale due to lack of appropriate comparator groups. Studies used heterogenous outcomes of clinical responses, angiographic stabilization, normalization of inflammatory markers, reduction in vascular uptake on positron emission tomography, reduction in prednisolone doses and relapses. Tocilizumab showed benefit in a RCT compared to placebo in a secondary per-protocol analysis but not the primary intention-to-treat analysis. Abatacept failed to demonstrate benefit compared to placebo for preventing relapses in another RCT. Pooled data from uncontrolled observational studies demonstrated beneficial clinical responses and angiographic stabilization in nearly 80% patients treated with tumour necrosis factor alpha inhibitors, tocilizumab or leflunomide. Certainty of evidence for outcomes from RCTs ranged from moderate to very low and was low to very low for all observational studies. There is a paucity of high-quality evidence to guide the pharmacotherapy of TAK. Future observational studies should attempt to include appropriate comparator arms. Multicentric, adequately powered RCTs assessing both clinical and angiographic responses are necessary in TAK.
Topics: Abatacept; Antibodies, Monoclonal; Antirheumatic Agents; Humans; Neoplasm Recurrence, Local; Randomized Controlled Trials as Topic; Takayasu Arteritis
PubMed: 33932173
DOI: 10.1007/s10067-021-05743-2 -
Archives of Cardiovascular Diseases May 2023Myocardial infarction is rare in children, teenagers and young adults (aged<20 years). The most common aetiologies identified include Kawasaki disease, familial... (Review)
Review
Myocardial infarction is rare in children, teenagers and young adults (aged<20 years). The most common aetiologies identified include Kawasaki disease, familial hypercholesterolaemia, collagen vascular disease-induced coronary arteritis, substance abuse (cocaine, glue sniffing), trauma, complications of congenital heart disease surgery, genetic disorders (such as progeria), coronary artery embolism, occult malignancy and several other rare conditions. Nephrotic syndrome is a very rare cause of myocardial infarction, but it is probably underestimated. The purpose of this review was to determine the current state of knowledge on acute coronary syndrome related to nephrotic syndrome. We thus performed a comprehensive structured literature search of the Medline database for articles published between January 1st, 1969 and December 31st, 2021. Myocardial infarction in young adults can be broadly divided into two groups: cases of angiographically normal coronary arteries; and cases of coronary artery disease of varying aetiology. There are several possible mechanisms underlying the association between acute coronary syndrome and nephrotic syndrome: (1) coronary thrombosis related to hypercoagulability and/or platelet hyperactivity; (2) atherosclerosis related to hyperlipidaemia; and (3) drug treatment. All of these mechanisms must be evaluated systematically in the acute phase of disease because they evolve rapidly with the treatment of nephrotic syndrome. In this review, we propose a decision algorithm for the management of acute coronary syndrome in the context of nephrotic syndrome. The final part of the review presents the short- and medium-term therapeutic strategies available. Thromboembolism related to nephrotic syndrome is a rare non-atherosclerotic cause of acute coronary syndrome, and prospective studies are needed to evaluate a systematic approach with personalized therapeutic strategies.
Topics: Humans; Adolescent; Young Adult; Child; Acute Coronary Syndrome; Nephrotic Syndrome; Myocardial Infarction; Coronary Artery Disease; Atherosclerosis
PubMed: 37088677
DOI: 10.1016/j.acvd.2023.03.002 -
The Permanente Journal Sep 2022IntroductionTakayasu's arteritis (TA) is an inflammatory condition that affects large vessels and frequently involves the aortic valve causing valve regurgitation.... (Review)
Review
IntroductionTakayasu's arteritis (TA) is an inflammatory condition that affects large vessels and frequently involves the aortic valve causing valve regurgitation. Surgical management is recommended for symptomatic severe aortic regurgitation (AR); however, the optimal surgical approach is yet unclear. This study aims to review surgical treatment options for AR in TA and determine which procedure has a lower chance of late postoperative events and/or mortality. MethodsAn electronic database search was performed within PubMed, EMBASE, Web of Science, and SCOPUS to identify articles from 1975 to 2016 focusing on surgical management of the AR in TA. ResultsTwenty seven studies encompassing a total of 194 cases (77% females) were included. Isolated aortic valve replacement (AVR) was performed in 105/194 cases (54%) (Group A), while combined aortic valve and root replacement (CAVRR) was performed in 87/194 (45%) (Group B). Prosthetic valve detachment was reported in 10/105 cases (9.5%) in group A and 1/87 cases (1.2%) in group B (p = 0.02). Dilation of the residual aorta was reported in 10/105 cases (9.5%) in group A and 1/87 cases (1.2%) in group B (p = 0.02). Any late (≥ 30 d) postoperative cardiac event was reported in 26/105 cases (24.8%) in group A, and in 7/87 cases (8.1%) in group B (p = 0.003). ConclusionsAlthough CAVRR is a more complex procedure, it might offer a better outcome in terms of late postoperative cardiac events compared to isolated AVR procedure. Future prospective studies are required to help determine the best surgical approach in such a population.
Topics: Aortic Valve; Aortic Valve Insufficiency; Female; Heart Valve Prosthesis Implantation; Humans; Male; Takayasu Arteritis
PubMed: 35939573
DOI: 10.7812/TPP/21.017