-
ACR Open Rheumatology Feb 2021Eosinophilic granulomatosis with polyangiitis (EGPA) is part of a group of vasculitides commonly referred to as antineutrophil cytoplasmic antibody (ANCA)-associated...
OBJECTIVE
Eosinophilic granulomatosis with polyangiitis (EGPA) is part of a group of vasculitides commonly referred to as antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV), in addition to granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and renal-limited vasculitis. Patients with EGPA characteristically have asthma and marked peripheral eosinophilia with only approximately 30% to 35% of patients being myeloperoxidase (MPO)-ANCA positive, distinguishing it from other forms of AAV (1,2). The aim of this systematic review is to support the development of the American College of Rheumatology/Vasculitis Foundation guideline for the management of EGPA.
METHODS
A systematic review was conducted of the literature for seven forms of primary systemic vasculitis (GPA, MPA, EGPA, polyarteritis nodosa, Kawasaki disease, giant cell arteritis, and Takayasu arteritis). The search was done for articles in English using Ovid Medline, PubMed, Embase, and the Cochrane Library. Articles were screened for suitability in addressing population/patients, intervention, comparator, and outcomes (PICO) questions, with studies presenting the highest level of evidence given preference. Two independent reviewers conducted a title/abstract screen and full-text review for each eligible study.
RESULTS
The initial search, conducted in August 2019, included 13 800 articles, of which 2596 full-text articles were reviewed. There were 190 articles (addressing 34 PICO questions) reporting on the diagnosis and management of EGPA.
CONCLUSION
This comprehensive systematic review synthesizes and evaluates the accuracy of commonly used tests for EGPA as well as benefits and toxicities of different treatment options.
PubMed: 33512787
DOI: 10.1002/acr2.11194 -
Clinical Rheumatology Jan 2022A systematic review and meta-analysis were conducted, according to the PRISMA methodology, to summarize current evidence on the prevalence and predictors of long-term... (Meta-Analysis)
Meta-Analysis Review
Long-term glucocorticoid treatment and high relapse rate remain unresolved issues in the real-life management of polymyalgia rheumatica: a systematic literature review and meta-analysis.
A systematic review and meta-analysis were conducted, according to the PRISMA methodology, to summarize current evidence on the prevalence and predictors of long-term glucocorticoid (GC) treatment and disease relapses in the real-life management of polymyalgia rheumatica (PMR).Out of 5442 retrieved studies, 21 were eligible for meta-analysis and 24 for qualitative analysis. The pooled proportions of patients still taking GCs at 1, 2, and 5 years were respectively 77% (95%CI 71-83%), 51% (95%CI 41-61%), and 25% (95CI% 15-36%). No significant difference was recorded by distinguishing study cohorts recruited before and after the issue of the international recommendations in 2010. The pooled proportion of patients experiencing at least one relapse at 1 year from treatment initiation was 43% (95%CI 29-56%). Female gender, acute-phase reactants levels, peripheral arthritis, starting GCs dosage, and tapering speed were the most frequently investigated potential predictors of prolonged GC treatment and relapse, but with inconsistent results. Only a few studies and with conflicting results evaluated the potential role of early treatment with methotrexate in reducing the GC exposure and the risk of relapse in PMR.This study showed that a high rate of prolonged GC treatment is still recorded in the management of PMR. The relapse rate, even remarkable, can only partially explain the long-term GC treatment, suggesting that other and not yet identified factors may be involved. Additional research is needed to profile patients with a higher risk of long-term GC treatment and relapse and identify more effective steroid-sparing strategies. Key Points: • High rate of long-term glucocorticoid (GC) treatment is recorded in polymyalgia rheumatica (PMR), being 77%, 51%, and 25% of patients still on GCs after respectively 1, 2, and 5 years. • A pooled relapse rate of 43% at 1 year, even remarkable, can only partially explain the long-term GC treatment in PMR. • Several studies have attempted to identify potential predictors of prolonged treatment with GCs and relapse, but with inconsistent results. • Additional research is needed to profile patients with a higher risk of long-term GC treatment and relapse and identify more effective steroid-sparing strategies.
Topics: Drug Administration Schedule; Female; Giant Cell Arteritis; Glucocorticoids; Humans; Polymyalgia Rheumatica; Recurrence
PubMed: 34415462
DOI: 10.1007/s10067-021-05819-z -
Medicine Apr 2015We aimed to clarify the role of 18F-fluorodeoxyglucose positron emission tomography (FDG-PET) in the management of large-vessel vasculitis (LVV), focusing on 3 issues... (Meta-Analysis)
Meta-Analysis Review
We aimed to clarify the role of 18F-fluorodeoxyglucose positron emission tomography (FDG-PET) in the management of large-vessel vasculitis (LVV), focusing on 3 issues which are as follows: describe and determine the different FDG-PET criteria for the diagnosis of vascular inflammation, the performance of FDG-PET for the diagnosis of large-vessel inflammation in giant cell arteritis (GCA) patients, and the performance of FDG-PET to evaluate the disease inflammatory activity in Takayasu arteritis (TA) patients. MEDLINE, Cochrane Library, and EMBASE database were searched for articles that evaluated the value of FDG-PET in LVV, from January 2000 to December 2013. Inclusion criteria were American College of Rheumatology criteria for GCA or TA, definition PET positivity threshold, and >4 cases included. Sensitivity (Se) and specificity (Sp) of FDG-PET for the diagnosis of large-vessel inflammation were calculated from each included individual study, and then pooled for meta-analysis with a random-effects model. Twenty-one studies (413 patients, 299 controls) were included in the systematic review. FDG-PET showed FDG vascular uptake in 70% (288/413) of patients and 7% (22/299) of controls. Only vascular uptake equal to or higher than the liver uptake was significantly different between GCA/TA patients and controls (P < 0.001). The meta-analysis of GCA patients (4 studies, 57 patients) shows that FDG-PET has high Se and Sp for the diagnosis of large-vessel inflammation in GCA patients in comparison to controls, with a pooled Se at 90% (95% confidence interval [CI], 79%-93%) and a pooled Sp at 98% (95% CI, 94%-99%). The meta-analysis of TA patients (7 studies, 191 patients) shows that FDG-PET has a pooled Se at 87% (95% CI, 78%-93%) and Sp at 73% (95% CI, 63%-81%) for the assessment of disease activity in TA, with up to 84% Sp, with studies using National Institutes of Health criteria as the disease activity assessment scale. FDG-PET showed good performances in the diagnosis of large-vessel inflammation, with higher accuracy in GCA patients than in TA patients. Although a vascular uptake equal to or higher than the liver uptake appears to be a good criterion for the diagnosis of vascular inflammation, further studies are needed to define the threshold of significance as well as the clinical significance of the vascular uptake.
Topics: Fluorodeoxyglucose F18; Giant Cell Arteritis; Humans; Positron-Emission Tomography; Radiopharmaceuticals; Takayasu Arteritis
PubMed: 25860208
DOI: 10.1097/MD.0000000000000622 -
International Journal of Environmental... Nov 2022Avascular osteonecrosis (AVN) is caused by the disrupted blood supply to the bone. Most AVN cases occur in the femoral head, but other sites might be affected as well,... (Meta-Analysis)
Meta-Analysis Review
Avascular osteonecrosis (AVN) is caused by the disrupted blood supply to the bone. Most AVN cases occur in the femoral head, but other sites might be affected as well, including the jaw or distal bones of the extremities. Previous studies suggested that diabetes could increase the risk of AVN of the jaw, but the relationship between diabetes and AVN in other bone sites is unclear. This systematic review and meta-analysis aimed to summarize the evidence from studies that had reported on the occurrence of AVN in sites other than the jaw, depending on the diagnosis of diabetes. Overall, we included 6 observational studies carried out in different populations: primary or secondary AVN of the femoral head, Takayasu arteritis, general population, kidney transplant recipients, systemic lupus erythematosus, and primary brain tumors. A random-effects meta-analysis showed that the risk of AVN in sites other than the jaw was non-significantly increased in patients with diabetes (odds ratio: 1.90, 95% confidence interval: 0.93-3.91). The pooled estimate increased and was significant after the exclusion of one study (2.46, 1.14-5.32). There was a significant heterogeneity (I = 65%, tau = 0.48, = 0.01; prediction interval, 0.21-16.84). There was no significant publication bias ( = 0.432). In conclusion, diabetes could increase the risk of AVN in sites other than the jaw, but the available evidence is limited. There is a need for large, well-designed, population-based studies.
Topics: Humans; Femur Head Necrosis; Diabetes Mellitus; Lupus Erythematosus, Systemic; Transplant Recipients; Odds Ratio
PubMed: 36429946
DOI: 10.3390/ijerph192215219 -
Seminars in Arthritis and Rheumatism Feb 2016Published small case series suggest that inflammatory bowel disease [IBD; Crohn's disease (CD) or ulcerative colitis (UC)] and vasculitis co-occur more frequently than... (Review)
Review
BACKGROUND
Published small case series suggest that inflammatory bowel disease [IBD; Crohn's disease (CD) or ulcerative colitis (UC)] and vasculitis co-occur more frequently than would be expected by chance.
OBJECTIVES
To describe this association by an analysis of a large cohort of carefully studied patients and through a systematic literature review.
METHODS
Patients with both IBD and vasculitis enrolled in the Vasculitis Clinical Research Consortium (VCRC) Longitudinal Studies, followed in Canadian Vasculitis research network (CanVasc) centers and/or in the University of Toronto's IBD clinic were included in this case series. A systematic literature review of patients with IBD and vasculitis involved a PubMed search through February 2014. The main characteristics of patients with Takayasu arteritis (TAK) and IBD were compared to those in patients with TAK without IBD followed in the VCRC.
RESULTS
The study identified 32 patients with IBD and vasculitis: 13 with large-vessel vasculitis [LVV; 12 with TAK, 1 with giant cell arteritis (GCA); 8 with CD, 5 with UC]; 8 with ANCA-associated vasculitis [AAV; 6 granulomatosis with polyangiitis (GPA), 2 with eosinophilic granulomatosis with polyangiitis (EGPA)]; 5 with isolated cutaneous vasculitis; and 6 with other vasculitides. Patients with LVV and AAV were mostly female (18/21). The diagnosis of IBD preceded that of vasculitis in 12/13 patients with LVV and 8/8 patients with AAV. The review of the literature identified 306 patients with IBD and vasculitis: 144 with LVV (133 TAK; 87 with IBD preceding LVV), 19 with AAV [14 GPA, 1 EGPA, 4 microscopic polyangiitis (MPA)], 66 with isolated cutaneous vasculitis, and 77 with other vasculitides. Patients with IBD and TAK were younger and had more frequent headaches, constitutional symptoms, or gastrointestinal symptoms compared to those patients in the VCRC who had TAK without IBD.
CONCLUSIONS
These findings highlight the risk of vasculitis, especially TAK, in patients with IBD (both CD and UC).
Topics: Female; Humans; Inflammatory Bowel Diseases; Male; Retrospective Studies; Vasculitis
PubMed: 26315859
DOI: 10.1016/j.semarthrit.2015.07.006 -
Clinical Neuroradiology Dec 2022Giant cell arteritis (GCA) is a systemic vasculitis that may cause ischemic stroke. Rarely, GCA can present with aggressive intracranial stenoses, which are refractory...
BACKGROUND
Giant cell arteritis (GCA) is a systemic vasculitis that may cause ischemic stroke. Rarely, GCA can present with aggressive intracranial stenoses, which are refractory to medical therapy. Endovascular treatment (EVT) is a possible rescue strategy to prevent ischemic complications in intracranial GCA but the safety and efficacy of EVT in this setting are not well-described.
METHODS
A systematic literature review was performed to identify case reports and series with individual patient-level data describing EVT for intracranial GCA. The clinical course, therapeutic considerations, and technique of seven endovascular treatments in a single patient from the authors' experience are presented.
RESULTS
The literature review identified 9 reports of 19 treatments, including percutaneous transluminal angioplasty (PTA) with or without stenting, in 14 patients (mean age 69.6 ± 6.3 years). Out of 12 patients 8 (66.7%) with sufficient data had > 1 pre-existing cardiovascular risk factor. All patients had infarction on MRI while on glucocorticoids and 7/14 (50%) progressed despite adjuvant immunosuppressive agents. Treatment was PTA alone in 15/19 (78.9%) cases and PTA + stent in 4/19 (21.1%). Repeat treatments were performed in 4/14 (28.6%) of patients (PTA-only). Non-flow limiting dissection was reported in 2/19 (10.5%) of treatments. The indications, technical details, and results of PTA are discussed in a single illustrative case. We report the novel use of intra-arterial calcium channel blocker infusion (verapamil) as adjuvant to PTA and as monotherapy, resulting in immediate improvement in cerebral blood flow.
CONCLUSION
Endovascular treatment, including PTA with or without stenting or calcium channel blocker infusion, may be effective therapies in medically refractory GCA with intracranial stenosis.
Topics: Humans; Middle Aged; Aged; Calcium Channel Blockers; Giant Cell Arteritis; Angioplasty; Stents; Constriction, Pathologic; Treatment Outcome; Angioplasty, Balloon
PubMed: 35503467
DOI: 10.1007/s00062-022-01171-0 -
European Journal of Nuclear Medicine... Jun 2021Polymyalgia rheumatica (PMR) can be difficult to diagnose. Whole-body [18F]FDG-PET/CT allows for a comprehensive evaluation of all relevant articular and extra-articular... (Meta-Analysis)
Meta-Analysis Review
PURPOSE
Polymyalgia rheumatica (PMR) can be difficult to diagnose. Whole-body [18F]FDG-PET/CT allows for a comprehensive evaluation of all relevant articular and extra-articular structures affected by PMR. We aimed to summarize current evidence on the diagnostic value of [18F]FDG-PET/CT for a diagnosis of PMR.
METHODS
PubMed/MEDLINE and the Cochrane Library database were searched from inception through May 31, 2020. Studies containing patients with PMR who underwent [18F]FDG-PET/CT were included. Screening and full-text review were performed by 3 investigators and data extraction by 2 investigators. Risk of bias was examined with the QUADAS-2 tool. Diagnostic test meta-analysis was performed with a bivariate model.
RESULTS
Twenty studies were included in the systematic review, of which 9 studies (n = 636 patients) were eligible for meta-analysis. [18F]FDG positivity at the following sites was associated with a diagnosis of PMR: interspinous bursae (positive likelihood ratio (LR+) 4.00; 95% CI 1.84-8.71), hips (LR+ 2.91; 95% CI 2.09-4.05), ischial tuberosities (LR+ 2.86; 95% CI 1.91-4.28), shoulders (LR+ 2.57; 95% CI 1.24-5.32) and sternoclavicular joints (LR+ 2.31; 95% CI 1.33-4.02). Negative likelihood ratios (LR-) for these sites, as well as the greater trochanters, were all less than 0.50. Composite [18F]FDG-PET/CT scores, as reported in 3 studies, provided a pooled LR+ of 3.91 (95% CI 2.42-6.32) and LR- of 0.19 (95% CI 0.10-0.36). Moderate to high heterogeneity was observed across the studies, mainly due to differences in patient selection, scanning procedures and/or interpretation criteria.
CONCLUSION
Significant [18F]FDG uptake at a combination of anatomic sites is informative for a diagnosis of PMR. [18F]FDG-PET/CT might be an important diagnostic tool in patients with suspected PMR. This study also highlights the need for adherence to published procedural recommendations and standardized interpretation criteria for the use of [18F]FDG-PET/CT in PMR.
Topics: Fluorodeoxyglucose F18; Giant Cell Arteritis; Humans; Polymyalgia Rheumatica; Positron Emission Tomography Computed Tomography; Positron-Emission Tomography; Radiopharmaceuticals
PubMed: 33372248
DOI: 10.1007/s00259-020-05162-6 -
Expert Review of Cardiovascular Therapy Oct 2010Reversible cerebral vasoconstriction syndrome (RCVS) is a cerebrovascular disorder associated with multifocal arterial constriction and dilation. RCVS is associated with... (Review)
Review
Reversible cerebral vasoconstriction syndrome (RCVS) is a cerebrovascular disorder associated with multifocal arterial constriction and dilation. RCVS is associated with nonaneurysmal subarachnoid hemorrhage, pregnancy and exposure to certain drugs. The primary clinical manifestation is recurrent sudden-onset and severe (‘thunderclap’) headaches over 1–3 weeks, often accompanied by nausea, vomiting, photophobia, confusion and blurred vision. The primary diagnostic dilemma is distinguishing RCVS from primary CNS arteritis. Diagnosis requires demonstration of the characteristic ‘string of beads’ on cerebral angiography with resolution within 1–3 months, although many patients will initially have normal vascular imaging. Many treatments have been reported to ameliorate the headaches of RCVS, but it is unclear whether they prevent hemorrhagic or ischemic complications.
Topics: Arteritis; Cerebral Angiography; Cerebral Arterial Diseases; Diagnosis, Differential; Female; Headache Disorders, Primary; Humans; Pregnancy; Syndrome; Time Factors; Vasoconstriction; Vasospasm, Intracranial
PubMed: 20936928
DOI: 10.1586/erc.10.124 -
Frontiers in Medicine 2022Chronic inflammation represents the cornerstone of the raised cardiovascular (CV) risk in patients with inflammatory rheumatic diseases (IRD), including vasculitis....
Chronic inflammation represents the cornerstone of the raised cardiovascular (CV) risk in patients with inflammatory rheumatic diseases (IRD), including vasculitis. Standardized mortality ratios in these patients are higher as compared to the general population, and the excess of premature mortality is due to early atherosclerotic events. Thus, IRD patients need appropriate CV risk assessment and management according to this CV disease (CVD) burden. Adequate control of CV risk is still lacking in usual care, but early diagnosis of silent and subclinical CVD is crucial to improve the long-term prognosis of these patients. Increased arterial stiffness may provide a pathophysiological link between inflammation and increased cardiovascular risk. Several noninvasive methods are now available to estimate artery stiffness in the clinical setting, including pulse wave velocity assessment. The independent predictive value of arterial stiffness for cardiovascular events has been demonstrated in general as well as in selected populations, and reference values adjusted for age and blood pressure have been suggested. Thus, arterial stiffness is an interesting biomarker for cardiovascular risk stratification. This systematic review summarizes the additional value that PWV measurement can provide in the setting of vasculitis, with a focus in the different clinical stages and CV risk prevention. This systematic review is registered with registration number: Prospero CRD42021259603.
PubMed: 35646970
DOI: 10.3389/fmed.2022.824630 -
Arthritis Research & Therapy Mar 2021Giant cell arteritis (GCA) is a common large vessel vasculitis in those over age 50 years. This meta-analysis examined the geographical and temporal distribution of... (Meta-Analysis)
Meta-Analysis
INTRODUCTION
Giant cell arteritis (GCA) is a common large vessel vasculitis in those over age 50 years. This meta-analysis examined the geographical and temporal distribution of the incidence, prevalence, and mortality of GCA.
METHODS
A systematic review was conducted using EMBASE, Scopus, and PubMed from their inceptions until 2019. Studies were included if they reported at least 50 or more GCA patients and defined the location and time frame. Articles on mortality were included and standardized mortality ratio (SMR) was extracted where possible. Mean pooled prevalence, incidence, and SMR were calculated using a random effects model. Linear regression was used to explore correlations between latitude and incidence, prevalence, and mortality.
RESULTS
Of the 3569 citations identified, 107 were included. The pooled incidence of GCA was 10.00 [9.22, 10.78] cases per 100,000 people over 50 years old. This incidence was highest in Scandinavia 21.57 [18.90, 24.23], followed by North and South America 10.89 [8.78, 13.00], Europe 7.26 [6.05, 8.47], and Oceania 7.85 [- 1.48, 17.19]. Pooled prevalence was 51.74 [42.04, 61.43] cases per 100,000 people over age 50. Annual mortality was 20.44 [17.84, 23.03] deaths/1000. Mortality generally decreased over the years of publication (p = 0.0008). Latitude correlated significantly with incidence (p = 0.0011), but not with prevalence, or mortality.
CONCLUSIONS
GCA incidence varies nearly 3-fold between regions and is highest in Scandinavia but not significantly. Mortality may be improving over time.
Topics: Europe; Giant Cell Arteritis; Humans; Incidence; Middle Aged; Prevalence; Scandinavian and Nordic Countries
PubMed: 33706808
DOI: 10.1186/s13075-021-02450-w