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RMD Open Jun 2023To identify criteria and descriptors used to measure response to treatment and change in disease activity in giant cell arteritis (GCA).
Measuring treatment outcomes and change in disease activity in giant cell arteritis: a systematic literature review informing the development of the EULAR-ACR response criteria on behalf of the EULAR-ACR response criteria in giant cell arteritis task force.
OBJECTIVES
To identify criteria and descriptors used to measure response to treatment and change in disease activity in giant cell arteritis (GCA).
METHODS
A systematic literature review (SLR) to retrieve randomised controlled trials (RCTs) and longitudinal observational studies (LOS). Criteria and descriptors of active disease, remission, response, improvement, worsening and relapse were extracted. RCTs, LOS with >20 subjects, and qualitative research studies were included.
RESULTS
10 593 studies were retrieved, of which 116 were included (11 RCTs, 104 LOS, 1 qualitative study). No unified definition of response to therapy was found. Most RCTs used composite endpoints to assess treatment outcomes. Active disease was described in all RCTs and 19% of LOS; and was largely defined by a combination of clinical and laboratory components. Remission was reported in 73% of RCTs and 42% of LOS; It was predominantly defined as the combination of clinical and laboratory components. One LOS reported response with a definition resembling the definition of remission from other studies. Improvement was rarely used as an endpoint and it was mostly a surrogate of remission. No study specifically defined worsening. Relapse was reported in all RCTs and 86% of LOS. It was predominantly defined as the combination of clinical, laboratory and treatment components.
CONCLUSIONS
The results of this SLR demonstrate that definitions of response used in clinical studies of GCA are scant and heterogeneous. RCTs and LOS mainly used remission and relapse as treatment outcomes. The descriptors identified will inform the development of the future European Alliance of Associations for Rheumatology-American College of Rheumatology response criteria for GCA.
Topics: Humans; United States; Giant Cell Arteritis; Outcome Assessment, Health Care; Treatment Outcome; Remission Induction; Recurrence
PubMed: 37349123
DOI: 10.1136/rmdopen-2023-003233 -
Immunoglobulin G4-related coronary periarteritis: a systematic literature review with a case series.Clinical Rheumatology Aug 2022We aimed to assess the clinical and radiological characteristics of immunoglobulin G4-related coronary periarteritis through a systematic literature review and from our... (Review)
Review
We aimed to assess the clinical and radiological characteristics of immunoglobulin G4-related coronary periarteritis through a systematic literature review and from our case series. In the systematic literature review, we assessed English language manuscripts on immunoglobulin G4-related coronary periarteritis cases. Additionally, we identified patients with immunoglobulin G4-related coronary periarteritis at St. Luke's International Hospital in Tokyo, Japan, from 2014 to 2020. We summarized patients' demographics, immunoglobulin-G and -G4 titers, site and morphological features of the coronary lesion, and other organ involvements. We identified 38 cases from the literature and four patients from our institute. Coronary lesions were detected using coronary computed tomography in 40 (95.2%) patients. Mass-like or diffuse wall-thickening lesion was the most frequently observed type in 33 (78.6%) patients. No trends at the site of the coronary arteries were identified. Overall, 32 (76.1%) patients had multiple-organ involvement, of which the most common lesion was peri-aortitis in 21 (50.0%) patients. Ten (23.8%) patients with an isolated coronary lesion had significantly lower immunoglobulin-G4 titers than those with other organ involvements (immunoglobulin-G4: 261 [161.0, 564.0] vs. 1355.0 [320.8, 2480.0] mg/dL, p = 0.033). The wall-thickening lesions responded well to immunosuppressive treatments. Mass-like or diffuse wall-thickening on coronary computed tomography is a characteristic radiographic finding of immunoglobulin G4-related coronary periarteritis, which can occur in any branch. Immunoglobulin G4-related coronary periarteritis showed similar characteristics to other organ lesions, including its relatively low serum immunoglobulin-G4 level in patients with a single-organ disease and its high responsiveness to glucocorticoids.
Topics: Aortitis; Arteritis; Coronary Vessels; Heart; Humans; Immunoglobulin G
PubMed: 35445950
DOI: 10.1007/s10067-022-06179-y -
Autoimmunity Reviews Mar 2023Takayasu arteritis (TAK) refractory to conventional disease-modifying anti-rheumatic drugs (DMARDs) is commonly treated with biologic DMARDs such as tocilizumab or tumor... (Meta-Analysis)
Meta-Analysis Review
Takayasu arteritis (TAK) refractory to conventional disease-modifying anti-rheumatic drugs (DMARDs) is commonly treated with biologic DMARDs such as tocilizumab or tumor necrosis factor-alpha inhibitors (TNFi). The 2021 American College of Rheumatology (ACR) recommendations preferred TNFi to tocilizumab. Therefore, we conducted a systematic review with meta-analysis to assess the evidence base for tocilizumab in TAK by updating a previous systematic review on DMARDs in TAK through searches on MEDLINE, Pubmed Central, Scopus, major international Rheumatology conference abstracts, and clinical trial databases from January 2021 to November 2022. Thirty-five studies involving 1082 TAK [one randomized controlled trial (RCT), eleven controlled and twenty-one uncontrolled studies, most of moderate to high quality] had evaluated tocilizumab in TAK. The RCT of tocilizumab versus placebo failed to meet its primary end-point of superiority of tocilizumab on an intention-to-treat analysis (hazard ratio 0.41, 95%CI 0.15-1.10) but successfully met the secondary end-point of superiority on per-protocol analysis (hazard ratio 0.34, 95%CI 0.11-1.00). A meta-analysis of six studies identified similar rates of clinical remission [risk ratio (RR) tocilizumab vs TNFi 1.03, 95%CI 0.91-1.17)], angiographic stabilization (RR 1.00, 95%CI 0.72-1.40) or adverse events (RR 0.84, 95%CI 0.54-1.31) with tocilizumab or TNFi. A meta-analysis of three studies identified superior clinical response (RR 1.55, 95%CI 1.15-2.10) and adverse effect profile (RR 0.45, 95%CI 0.25-0.80) with tocilizumab than cyclophosphamide. Pooled data from uncontrolled studies identified clinical response in 85%(95%CI 79-91%) and angiographic stabilization in 82% (95%CI 68-94%). Our study suggests similar evidence for treating TAK with tocilizumab or TNFi, contrary to the ACR 2021 recommendations.
Topics: Humans; Tumor Necrosis Factor-alpha; Takayasu Arteritis; Antibodies, Monoclonal, Humanized; Antirheumatic Agents; Immunologic Factors
PubMed: 36652977
DOI: 10.1016/j.autrev.2023.103275 -
International Journal of Rheumatic... Jul 2023The aim of this meta-analysis is to investigate the relationship between interleukin (IL)-10 levels and its polymorphism and Takayasu arteritis (TAK). (Meta-Analysis)
Meta-Analysis
OBJECTIVE
The aim of this meta-analysis is to investigate the relationship between interleukin (IL)-10 levels and its polymorphism and Takayasu arteritis (TAK).
METHODS
Five databases including PubMed, Web of Science, Ovid, Sinomed and China National Knowledge Infrastructure (CNKI) were gone through from inception to March 31, 2022. Studies were screened according to the inclusion and exclusion criteria. Newcastle-Ottawa Scale (NOS) was applied to assess study quality. Strengths of association were evaluated by odds ratio (OR) and 95% CI. The T v. t (allele contrast), TT v. tt (homozygous contrast), Tt vs tt (heterozygous contrast), TT + Tt vs tt (dominant contrast) and TT vs Tt + tt (recessive contrast) models were adopted.
RESULTS
Seven studies were included. No significant relationship between IL-10 and TAK was detected in the included patients (P > 0.05). The levels of IL-10 were lower in the active group than those in the stable group, which was -0.47 (95% CI: -0.93, 0.00) (P = 0.05). No significant relationships between IL-10 and TAK were found under all contrasts for polymorphisms rs1800871, rs1800872 and rs1800896 (P > 0.05).
CONCLUSIONS
There was no significant difference in IL-10 levels between TAK patients and control subjects. The levels of IL-10 were lower in TAK patients in the active stage. There was no significant association between IL-10 gene polymorphisms and TAK. Further well-designed studies with larger sample sizes in patients with different stages are needed.
Topics: Humans; Interleukin-10; Takayasu Arteritis; Genetic Predisposition to Disease; Polymorphism, Genetic; China; Polymorphism, Single Nucleotide
PubMed: 37137733
DOI: 10.1111/1756-185X.14717 -
Zeitschrift Fur Rheumatologie Feb 2024This study aimed to update the prevalence estimates of inflammatory rheumatic diseases (IRD) in Germany.
OBJECTIVE
This study aimed to update the prevalence estimates of inflammatory rheumatic diseases (IRD) in Germany.
METHODS
A systematic literature search in PubMed and Web of Science (last search 08 November 2022) identified original articles (regional and nationwide surveys and claims data analyses for arthritides, connective tissue diseases, and vasculitides) on prevalences for the period 2014-2022. Data sources, collection period, case definition, and risk of bias are reported. Prevalences were estimated from available national data, with consideration of international data.
RESULTS
Screening by two authors yielded 263 hits, of which 18 claims data analyses and 2 surveys met the inclusion criteria. Prevalences 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% (Sjögren'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. The prevalence of juvenile idiopathic arthritis was reported to be around 0.10% (0.07-0.10%) of 0-18-year-olds, 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 claims data analyses. In the absence of multistage population studies, the available data are, overall, uncertain sources for prevalence estimates, with a moderate to high risk of bias.
Topics: Child; Adolescent; Humans; Prevalence; Arthritis, Rheumatoid; Spondylitis, Ankylosing; Polymyalgia Rheumatica; Sjogren's Syndrome; Lupus Erythematosus, Systemic; Rheumatic Fever; Giant Cell Arteritis; Rheumatic Diseases
PubMed: 36749363
DOI: 10.1007/s00393-022-01302-5 -
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 -
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 -
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 -
Seminars in Arthritis and Rheumatism Oct 2022To conduct a systematic literature review and meta-analysis to estimate the proportion of fever of unknown origin (FUO) and inflammation of unknown origin (IUO) cases... (Meta-Analysis)
Meta-Analysis Review
OBJECTIVES
To conduct a systematic literature review and meta-analysis to estimate the proportion of fever of unknown origin (FUO) and inflammation of unknown origin (IUO) cases that are due to rheumatic disorders and the relative frequency of specific entities associated with FUO/IUO.
METHODS
We searched PubMed and EMBASE between January 1, 2002, and December 31, 2021, for studies with ≥50 patients reporting on causes of FUO/IUO. The primary outcome was the proportion of FUO/IUO patients with rheumatic disease. Secondary outcomes include the association between study and patient characteristics and the proportion of rheumatic disease in addition to the relative frequency of rheumatic disorders within this group. Proportion estimates were calculated using random-effects models.
RESULTS
The included studies represented 16884 patients with FUO/IUO. Rheumatic disease explained 22.2% (95%CI 19.6 - 25.0%) of cases. Adult-onset Still's disease (22.8% [95%CI 18.4-27.9%]), giant cell arteritis (11.4% [95%CI 8.0-16.3%]), and systemic lupus erythematosus (11.1% [95%CI 9.0-13.8%]) were the most frequent disorders. The proportion of rheumatic disorders was significantly higher in high-income countries (25.9% [95%CI 21.5 - 30.8%]) versus middle-income countries (19.5% [95%CI 16.7 - 22.7%]) and in prospective studies (27.0% [95%CI 21.9-32.8%]) versus retrospective studies (20.6% [95%CI 18.1-24.0%]). Multivariable meta-regression analysis demonstrated that rheumatic disease was associated with the fever duration (0.011 [95%CI 0.003-0.021]; P=0.01) and with the fraction of patients with IUO (1.05 [95%CI 0.41-1.68]; P=0.002).
CONCLUSION
Rheumatic disorders are a common cause of FUO/IUO. The care of patients with FUO/IUO should involve physicians who are familiar with the diagnostic workup of rheumatic disease.
Topics: Adult; Fever of Unknown Origin; Fluorodeoxyglucose F18; Humans; Inflammation; Prospective Studies; Retrospective Studies; Rheumatic Diseases
PubMed: 35868032
DOI: 10.1016/j.semarthrit.2022.152066 -
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