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PloS One 2021Takayasu arteritis (TAK) is a rare immune-mediated vasculitis of the aorta and its branches. Aims were to calculate prevalence and incidence in Switzerland, to assess...
OBJECTIVE
Takayasu arteritis (TAK) is a rare immune-mediated vasculitis of the aorta and its branches. Aims were to calculate prevalence and incidence in Switzerland, to assess disease activity and performance of MR-Angiography (MRA).
METHODS
31 patients were recorded in a database, 27 were followed prospectively up to 3 years. Prevalence was calculated based on data of the national statistical bureau. Disease activity was defined using the revised EULAR criteria. MRA depicted stenotic changes and aortic wall enhancement.
RESULTS
A disease prevalence of 14.5/1.000.000 inhabitants and an incidence of 0.3/1.000.000 per year was calculated. Aortic wall enhancement was found in 10 patients while in clinical and serological remission. EULAR criteria missed 5 patients with disease activity with isolated elevations of ESR/CRP. Arterial stenosis did not change over time in 5 cases, it improved in 2 and increased in 7. At follow-up 16 patients were treated with tocilizumab, 11/16 in monotherapy, 5 patients were treatment-free, 25/27 stayed in remission.
CONCLUSION
In addition to prevalence and incidence, our data show that MRA qualifies to detect subclinical disease activity, but, on the other hand, that EULAR criteria may miss disease activity in case of isolated elevation of ESR/CRP.
Topics: Adolescent; Adult; Aged; Aged, 80 and over; Child; Child, Preschool; Female; Follow-Up Studies; Humans; Magnetic Resonance Angiography; Male; Middle Aged; Prevalence; Prognosis; Prospective Studies; Switzerland; Takayasu Arteritis; Tomography, X-Ray Computed; Young Adult
PubMed: 34143786
DOI: 10.1371/journal.pone.0250025 -
Internal Medicine (Tokyo, Japan) Apr 2023A 77-year-old man arrived at our hospital with bilateral shoulder pain persisting for several months and headache for 1 month. Giant cell arteritis with polymyalgia...
A 77-year-old man arrived at our hospital with bilateral shoulder pain persisting for several months and headache for 1 month. Giant cell arteritis with polymyalgia rheumatica was suspected. However, considering his medical history of testing positive for syphilis, we submitted a sample for a syphilis serology test, which yielded positive results. The Treponema pallidum hemagglutination assay of cerebrospinal fluid was positive, and a temporal artery biopsy revealed vasculitis, confirming the diagnosis of tertiary syphilis. He was successfully treated for two weeks with penicillin G infusions. Symptoms reminiscent of giant cell arteritis and polymyalgia rheumatica may reveal syphilis, which is called the "great imitator."
Topics: Male; Humans; Aged; Giant Cell Arteritis; Polymyalgia Rheumatica; Syphilis; Treponema pallidum; Temporal Arteries
PubMed: 36047113
DOI: 10.2169/internalmedicine.9779-22 -
RMD Open Feb 2022Despite the heterogeneity of the giant cell arteritis (GCA) at the level of clinical manifestations and the cellular and molecular players involved in its pathogenesis,... (Review)
Review
Despite the heterogeneity of the giant cell arteritis (GCA) at the level of clinical manifestations and the cellular and molecular players involved in its pathogenesis, GCA is still treated with standardised regimens largely based on glucocorticoids (GC). Long-term use of high dosages of GC as required in GCA are associated with many clinically relevant side effects. In the recent years, the interleukin-6 receptor blocker tocilizumab has become available as the only registered targeted immunosuppressive agent in GCA. However, immunological heterogeneity may require different pathways to be targeted in order to achieve a clinical, immunological and vascular remission in GCA. The advances in the targeted blockade of various molecular pathways involved in other inflammatory and autoimmune diseases have catalyzed the research on targeted therapy in GCA. This article gives an overview of the studies with targeted immunosuppressive treatments in GCA, with a focus on their clinical value, including their effects at the level of vascular inflammation.
Topics: Giant Cell Arteritis; Glucocorticoids; Humans; Immunosuppression Therapy; Immunosuppressive Agents
PubMed: 35149602
DOI: 10.1136/rmdopen-2021-001652 -
Autoimmunity Reviews Jan 2024Giant cell arteritis (GCA) and polymyalgia rheumatica (PMR) are common conditions in older adults. Their clinical connection has been recognized over time, with many... (Review)
Review
Giant cell arteritis (GCA) and polymyalgia rheumatica (PMR) are common conditions in older adults. Their clinical connection has been recognized over time, with many patients experiencing both conditions separately, simultaneously or in temporal sequence to each other. Early GCA detection is essential to prevent vascular damage, but identifying subclinical GCA in PMR patients remains a challenge and routine screening is not standard practice. Subclinical GCA prevalence in newly diagnosed PMR patients ranges from 23 to 29%, depending on the screening method. Vessel wall imaging and temporal artery biopsy can detect subclinical GCA. Epidemiology and trigger factors show similarities between the two conditions, but PMR is more common than GCA. Genetic and pathogenesis studies reveal shared inflammatory mechanisms involving dendritic cells, pro-inflammatory macrophages, and an IL-6 signature. However, the inflammatory infiltrates differ, with extensive T cell infiltrates seen in GCA while PMR shows an incomplete profile of T cell and macrophage-derived cytokines. Glucocorticoid treatment is effective for both conditions, but the steroid requirements vary. PMR overall mortality might be similar to the general population, while GCA patients with aortic inflammatory aneurysms face increased mortality risk. The GCA-PMR association warrants further research. Considering their kinship, recently the term GCA-PMR Spectrum Disease (GPSD) has been proposed.
Topics: Humans; Aged; Giant Cell Arteritis; Polymyalgia Rheumatica; Glucocorticoids
PubMed: 37625672
DOI: 10.1016/j.autrev.2023.103415 -
Journal of the American Society of... Sep 2021Renal involvement in ANCA-associated vasculitis (AAV) is associated with poor outcomes. The clinical significance of arteritis of the small kidney arteries has not been...
BACKGROUND
Renal involvement in ANCA-associated vasculitis (AAV) is associated with poor outcomes. The clinical significance of arteritis of the small kidney arteries has not been evaluated in detail.
METHODS
In a multicenter cohort of patients with AAV and renal involvement, we sought to describe the clinicopathologic characteristics of patients with AAV who had renal arteritis at diagnosis, and to retrospectively analyze their prognostic value.
RESULTS
We included 251 patients diagnosed with AAV and renal involvement between 2000 and 2019, including 34 patients (13.5%) with arteritis. Patients with AAV-associated arteritis were older, and had a more pronounced inflammatory syndrome compared with patients without arteritis; they also had significantly lower renal survival (=0.01). In multivariable analysis, the ANCA renal risk score, age at diagnosis, history of diabetes mellitus, and arteritis on index kidney biopsy were independently associated with ESKD. The addition of the arteritis status significantly improved the discrimination of the ANCA renal risk score, with a concordance index (C-index) of 0.77 for the ANCA renal risk score alone, versus a C-index of 0.80 for the ANCA renal risk score plus arteritis status (=0.008); ESKD-free survival was significantly worse for patients with an arteritis involving small arteries who were classified as having low or moderate risk, according to the ANCA renal risk score. In two external validation cohorts, we confirmed the incidence and phenotype of this AAV subtype.
CONCLUSIONS
Our findings suggest AAV with renal arteritis represents a different subtype of AAV with specific clinical and histologic characteristics. The prognostic contribution of the arteritis status remains to be prospectively confirmed.
Topics: Aged; Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis; Arteritis; Disease-Free Survival; Female; France; Humans; Kidney Failure, Chronic; Male; Middle Aged; Prognosis; Renal Artery; Retrospective Studies; Risk Factors
PubMed: 34155059
DOI: 10.1681/ASN.2020071074 -
Women's Health (London, England) 2023Giant cell arteritis (GCA) and polymyalgia rheumatica (PMR) are two common systemic inflammatory conditions with a combined lifetime risk of approximately 3.5% in women... (Review)
Review
Giant cell arteritis (GCA) and polymyalgia rheumatica (PMR) are two common systemic inflammatory conditions with a combined lifetime risk of approximately 3.5% in women and 1.5% in men. They are intimately associated with the aging process, virtually never occurring prior to 50 years of age and becoming more common over time. The reasons for this are unclear, but likely relate in part to factors related to aging of the immune system. The treatment of both GCA and PMR is traditionally based on glucocorticoids, frequently requiring a prolonged treatment course over long periods of time. Other medications are belatedly entering our treatment armamentarium, but their exact place in treatment algorithms remains to be fully defined and it is likely glucocorticoids will remain a cornerstone of our treatment in GCA and PMR for the foreseeable future. As a result, people with GCA and PMR will continue to be exposed to a significant cumulative glucocorticoid burden with all of the attendant potential adverse events, including osteoporosis. The predominantly post-menopausal female population that most commonly develops PMR and GCA is also the population that is most affected by osteoporosis. Given the risk of glucocorticoid-induced osteoporosis and subsequent fragility fractures, a planned treatment approach from glucocorticoid initiation is needed in these conditions. For the majority of patients, this will entail ensuring sufficiency of calcium and vitamin D as well as antiresorptive treatments. In this article, we discuss considerations around optimisation of metabolic bone health in GCA and PMR.
Topics: Male; Humans; Female; Giant Cell Arteritis; Polymyalgia Rheumatica; Glucocorticoids; Bone Density; Osteoporosis
PubMed: 36627860
DOI: 10.1177/17455057221147385 -
RMD Open Apr 2023To assess outcomes in giant cell arteritis (GCA) patients during and after long-term tocilizumab (TCZ) treatment.
OBJECTIVE
To assess outcomes in giant cell arteritis (GCA) patients during and after long-term tocilizumab (TCZ) treatment.
METHODS
Retrospective analysis of GCA patients treated with TCZ at a single centre (2010-2022). Time to relapse and annualised relapse rate during and after TCZ treatment, prednisone use, and safety were assessed. Relapse was defined as reappearance of any GCA clinical manifestation that required treatment intensification, regardless of C reactive protein levels and erythrocyte sedimentation rate.
RESULTS
Sixty-five GCA patients were followed for a mean (SD) of 3.1 (1.6) years. The mean duration of the initial TCZ course was 1.9 (1.1) years. The Kaplan-Meier (KM)-estimated relapse rate at 18 months on TCZ was 15.5%. The first TCZ course was discontinued due to satisfactory remission achievement in 45 (69.2%) patients and adverse events in 6 (9.2%) patients. KM-estimated relapse rate at 18 months after TCZ discontinuation was 47.3%. Compared with patients stopping TCZ at or before 12 months of treatment, the multivariable adjusted HR (95% CI) for relapse in patients on TCZ beyond 12 months was 0.01 (0.00 to 0.28; p=0.005). Thirteen patients received >1 TCZ course. Multivariable adjusted annualised relapse rates (95% CI) in all periods on and off TCZ aggregated were 0.1 (0.1 to 0.2) and 0.4 (0.3 to 0.7), respectively (p=0.0004). Prednisone was discontinued in 76.9% of patients. During the study, 13 serious adverse events occurred in 11 (16.9%) patients.
CONCLUSION
Long-term TCZ treatment was associated with remission maintenance in most patients with GCA. The estimated relapse rate by 18 months after TCZ discontinuation was 47.3%.
Topics: Humans; Giant Cell Arteritis; Prednisone; Retrospective Studies; Treatment Outcome; Recurrence
PubMed: 37024237
DOI: 10.1136/rmdopen-2022-002923 -
WMJ : Official Publication of the State... Mar 2023Temporal artery biopsy is ordered when clinical symptoms and an elevated C-reactive protein values and/or erythrocyte sedimentation rates suggest giant cell arteritis....
BACKGROUND
Temporal artery biopsy is ordered when clinical symptoms and an elevated C-reactive protein values and/or erythrocyte sedimentation rates suggest giant cell arteritis. The percentage of temporal artery biopsies positive for giant cell arteritis is low. The objectives of our study were to analyze the diagnostic yield of temporal artery biopsies at an independent academic medical center and to develop a risk stratification model for triaging patients for possible temporal artery biopsy.
METHODS
We retrospectively reviewed the electronic health records of all patients who underwent temporal artery biopsy in our institution from January 2010 through February 2020. We compared clinical symptoms and inflammatory marker (C-reactive protein and erythrocyte sedimentation rate) values of patients whose specimens were positive for giant cell arteritis with those of patients with negative specimens. Statistical analysis included descriptive statistics, chi-square test, and multivariable logistic regression. A risk stratification tool, which included point assignments and measures of performance, was developed.
RESULTS
Of 497 temporal artery biopsies for giant cell arteritis performed, 66 were positive and 431 were negative. Jaw/tongue claudication, elevated inflammatory marker values, and age were associated with a positive result. Using our risk stratification tool, 3.4% of low-risk patients, 14.5% of medium-risk patients, and 43.9% of high-risk patients were positive for giant cell arteritis.
CONCLUSIONS
Jaw/tongue claudication, age, and elevated inflammatory markers were associated with positive biopsy results. Our diagnostic yield was much lower when compared with a benchmark yield determined in a published systematic review. A risk stratification tool was developed based on age and the presence of independent risk factors.
Topics: Humans; Biopsy; C-Reactive Protein; Giant Cell Arteritis; Headache; Retrospective Studies; Temporal Arteries
PubMed: 36940120
DOI: No ID Found -
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 -
RMD Open Feb 2024Relapses and late complications remain a concern in giant cell arteritis (GCA). Monitoring strategies are required to effectively tailor treatment and improve patients'...
Relapses and late complications remain a concern in giant cell arteritis (GCA). Monitoring strategies are required to effectively tailor treatment and improve patients' outcomes. Current monitoring of GCA is based on clinical assessment and evaluation of traditional inflammatory markers such as C reactive protein and erythrocyte sedimentation rate; however, this approach has limited value in patients receiving interleukin (IL)-6 blocking agents. New blood biomarkers that are less dependent on the IL-6 axis such as IL-23, B cell activating factor, osteopontin and calprotectin have been explored, but none of them has yet accumulated sufficient evidence to qualify as a routine follow-up parameter. Imaging techniques, including ultrasound and 18F-fluorodeoxyglucose positron emission tomography/computed tomography, potentially offer additional insights; however, the choice of the imaging method as well as its interpretation must be investigated further. Future studies are required to investigate the outcome of patients with GCA whose treatment decisions are based on traditional plus novel (laboratory and imaging) biomarkers as compared with those undergoing conventional monitoring strategies.
Topics: Humans; Giant Cell Arteritis; Diagnostic Imaging; Positron Emission Tomography Computed Tomography; Glucocorticoids; Biomarkers; Interleukin-6
PubMed: 38395453
DOI: 10.1136/rmdopen-2023-003397