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Innere Medizin (Heidelberg, Germany) Feb 2024Giant cell arteritis (GCA) and Takayasu arteritis (TAK), as the main representatives of large vessel vasculitis, are rheumatological autoimmune disorders associated with... (Review)
Review
BACKGROUND
Giant cell arteritis (GCA) and Takayasu arteritis (TAK), as the main representatives of large vessel vasculitis, are rheumatological autoimmune disorders associated with inflammatory vessel wall changes in the arterial system that can lead to many types of organ damage.
MATERIAL AND METHODS
In this review the current scientific evidence on the diagnostics and treatment of large vessel vasculitis is evaluated and discussed.
RESULTS
In addition to the medical history and clinical presentation, imaging techniques nowadays represent the core of large vessel vasculitis diagnostics and have largely replaced the histological confirmation of GCA. After the diagnosis, acute treatment with glucocorticoids should be initiated as rapidly as possible but in the long term this should be tapered out or replaced by a steroid-sparing basic treatment. In contrast to GCA with already available options and other biologic disease-modifying antirheumatic drugs (DMARDs) about to be approved, there are still no approved biologic DMARD treatment options available for the less common TAK.
CONCLUSION
In contrast to the substantial progress in imaging diagnostics of large vessel vasculitis and with respect to the treatment of GCA, the much rarer TAK still requires intensive research efforts, especially to improve the treatment situation.
Topics: Humans; Giant Cell Arteritis; Takayasu Arteritis; Glucocorticoids; Antirheumatic Agents; Biological Products
PubMed: 38240814
DOI: 10.1007/s00108-023-01656-1 -
Rheumatology (Oxford, England) May 2024This study aimed to compare the efficacy and safety of adalimumab (ADA) vs tocilizumab (TCZ) in patients with Takayasu arteritis (TAK). (Randomized Controlled Trial)
Randomized Controlled Trial Comparative Study
OBJECTIVE
This study aimed to compare the efficacy and safety of adalimumab (ADA) vs tocilizumab (TCZ) in patients with Takayasu arteritis (TAK).
METHODS
This was a randomized, controlled, open-label study. Forty patients with active and severe TAK were enrolled. They were treated with ADA (n = 21) combined with glucocorticoids (GCs) and MTX or TCZ (n = 19) combined with GCs and MTX. The planned follow-up duration was 12 months. The primary end point was the efficacy rate (ER) at 6 months. The secondary end points included ER at 9 and 12 months, relapse rate, GC tapering, adverse effects, and life quality changes during treatment.
RESULTS
In the intention-to-treat (ITT) population, the ER at 6 months was higher in the ADA group (85.71% vs 52.63%, P = 0.02). A similar direction of effect was noted in the per-protocol set (89.47% vs 62.50%, P = 0.06). The percentages of patients who achieved a GC dose of ≤10 mg/day at 6 months were similar between the ADA and TCZ groups (47.37% vs 43.75%, P = 0.83). The ERs at 9 and 12 months were similar between the two groups (P > 0.05). During the first 12 months of treatment, the relapse rate and adverse event incidence were comparable between the two groups (ADA vs TCZ: 9.52% vs 10.53%, P = 0.96; 38.10% vs 47.37%, P = 0.55, respectively).
CONCLUSION
ADA combined with GCs and MTX may be more efficacious than TCZ combined with GCs and MTX among patients with active and severe TAK.
TRIAL REGISTRATION
Clinicaltrials.gov; NCT04300686.
Topics: Humans; Takayasu Arteritis; Female; Adult; Antibodies, Monoclonal, Humanized; Adalimumab; Male; Treatment Outcome; Drug Therapy, Combination; Glucocorticoids; Methotrexate; Young Adult; Severity of Illness Index; Antirheumatic Agents; Middle Aged; Quality of Life
PubMed: 37540159
DOI: 10.1093/rheumatology/kead387 -
The Journal of Rheumatology Oct 2023To evaluate the safety and efficacy of tocilizumab (TCZ) in giant cell arteritis (GCA) in a large North American cohort.
OBJECTIVE
To evaluate the safety and efficacy of tocilizumab (TCZ) in giant cell arteritis (GCA) in a large North American cohort.
METHODS
Patients with GCA treated with TCZ between January 1, 2010, and May 15, 2020, were retrospectively identified. Kaplan-Meier methods were used to estimate time to TCZ discontinuation and time to first relapse after TCZ discontinuation. Poisson regression models were used to compare annualized relapse rates before, during, and after TCZ use. Age- and sex-adjusted risk factors associated with relapse on and off TCZ and development of adverse events of significant interest (AESIs) were examined using Cox models.
RESULTS
One hundred fourteen patients (60.5% female) were included with mean (SD) age 70.4 (8.2) years. Median duration from GCA diagnosis to TCZ start was 4.5 months. Median overall duration of TCZ treatment was 2.3 years. Relapse rate prior to TCZ start (0.84 relapses/person-year) was 3-fold reduced while on TCZ (0.28 relapses/person-year; < 0.001) but increased to 0.64 relapses/person-year after TCZ discontinuation. Fifty-two patients stopped TCZ after a median of 16.8 months; 27 relapsed after discontinuation (median: 8.4 months; 58% relapsed within 12 months). Only 14.9% of patients stopped TCZ because of AESIs. Neither dose/route of TCZ, presence of large-vessel vasculitis, nor duration of TCZ therapy prior to discontinuation predicted relapse after TCZ stop.
CONCLUSION
TCZ is well tolerated in GCA, with low rates of discontinuation for AESIs. However, relapse occurred in > 50% despite median treatment > 12 months. Since the duration of TCZ prior to discontinuation did not significantly affect subsequent risk of GCA recurrence, further research is needed to determine the optimal duration of therapy.
Topics: Humans; Female; Aged; Male; Giant Cell Arteritis; Cohort Studies; Retrospective Studies; Treatment Outcome; Recurrence
PubMed: 37321636
DOI: 10.3899/jrheum.2022-1214 -
Rheumatology (Oxford, England) Jan 2024The main objective of this study was to analyse the prevalence and characteristics of subclinical GCA in patients with PMR.
OBJECTIVE
The main objective of this study was to analyse the prevalence and characteristics of subclinical GCA in patients with PMR.
METHODS
This was a cross-sectional multicentre international study of consecutive patients with newly diagnosed PMR without symptoms or signs suggestive of GCA. All patients underwent US of the temporal superficial, common carotid, subclavian and axillary arteries. Patients with halo signs in at least one examined artery were considered to have subclinical GCA. The clinical, demographic and laboratory characteristics of the PMR group without subclinical vasculitis were compared with subclinical GCA, and the pattern of vessel involvement was compared with that of a classical single-centre GCA cohort.
RESULTS
We included 346 PMR patients, 267 (77.2%) without subclinical GCA and 79 (22.8%) with subclinical GCA. The PMR patients with subclinical GCA were significantly older, had a longer duration of morning stiffness and more frequently reported hip pain than PMR without subclinical GCA. PMR with subclinical GCA showed a predominant extracranial large vessel pattern of vasculitic involvement compared with classical GCA, where the cranial phenotype predominated. The patients with PMR in the classical GCA group showed a pattern of vessel involvement similar to classical GCA without PMR but different from PMR with subclinical involvement.
CONCLUSION
More than a fifth of the pure PMR patients had US findings consistent with subclinical GCA. This specific subset of patients showed a predilection for extracranial artery involvement. The optimal screening strategy to assess the presence of vasculitis in PMR remains to be determined.
Topics: Humans; Giant Cell Arteritis; Polymyalgia Rheumatica; Prevalence; Cross-Sectional Studies; Pain
PubMed: 37129541
DOI: 10.1093/rheumatology/kead189 -
Clinical and Experimental Rheumatology Apr 2024Neurosensory hearing loss is well-documented in chronic autoimmune conditions such as systemic lupus erythematosus (SLE). However, the literature lacks data on the...
OBJECTIVES
Neurosensory hearing loss is well-documented in chronic autoimmune conditions such as systemic lupus erythematosus (SLE). However, the literature lacks data on the prevalence and characteristics of hearing impairment in Takayasu's arteritis (TAK). In this cross-sectional study, our principal objective was to systematically assess the auditory function of individuals diagnosed with TAK, against SLE patients and healthy controls (HC).
METHODS
Age and gender matched TAK and SLE patients followed up in a tertiary centre along with healthy controls were included in a two-phase study. In the first phase, a questionnaire on ENT symptoms was administered to the patient (TAK: n=104 and SLE: n= 151) and HC (n=174) groups. In the second phase, patients (TAK: n=53 and SLE: n=33) and HC (n=45) underwent audiometric tests.
RESULTS
The questionnaire survey revealed that both TAK and SLE patients reported hearing loss (27.9%, 25.8%, 7.4%, p<0.001), tinnitus (49%, 35.8%, 13.8%, p<0.001) and vertigo (46.2%, 33.8%, 16.7%, p<0.001) at significantly higher rates than HC. Audiometry results indicated that both TAK (30.2%) and SLE patients (18.2%) had increased hearing loss compared to HC (8.9%), however, only TAK patients were found to have significantly increased risk in age adjusted logistic regression analysis (OR= 3.915, 95%CI: 1.179-12.998, p=0.026). Hearing loss was mainly neurosensory in all groups. TAK patients were affected at both low (<6000 Hz) and high (>6000 Hz) frequencies, whereas SLE patients were affected only at high frequencies. Hearing loss was significantly associated only with older age. No association was observed with the anatomical location of vascular involvement or history of stroke.
CONCLUSIONS
Our study reveals an increased prevalence of hearing loss in TAK. Further research is crucial to uncover the underlying causes.
Topics: Humans; Takayasu Arteritis; Female; Male; Adult; Cross-Sectional Studies; Lupus Erythematosus, Systemic; Prevalence; Middle Aged; Tinnitus; Surveys and Questionnaires; Case-Control Studies; Vertigo; Risk Factors; Hearing Loss; Young Adult; Logistic Models; Tertiary Care Centers; Hearing; Audiometry; Odds Ratio
PubMed: 38489315
DOI: 10.55563/clinexprheumatol/gtd2uv -
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 -
Clinical Rheumatology Oct 2023
Topics: Humans; Giant Cell Arteritis; Temporal Arteries; Arteries; Chronic Disease; Recurrence
PubMed: 37405527
DOI: 10.1007/s10067-023-06691-9 -
Rheumatology (Oxford, England) Sep 2023Takayasu arteritis (TAK) is a large-vessel vasculitis rarely reported in children and infants. Most articles on paediatric TAK have not focused on infants. We present...
OBJECTIVES
Takayasu arteritis (TAK) is a large-vessel vasculitis rarely reported in children and infants. Most articles on paediatric TAK have not focused on infants. We present the largest case series of infantile TAK, aiming to identify its demographic and clinical characteristics and compare them with existing data on older children.
METHODS
We conducted an international multicentre retrospective cohort study. Epidemiological and clinical data were collected from patients' charts from six rheumatology centres. All patients met both the EULAR/PReS 2008 criteria and the 1990 ACR/EULAR criteria and were diagnosed with TAK at age <5 years.
RESULTS
Twelve patients were included (50% female). Median age of symptom onset was 11 months, with a diagnostic delay of 4 months. The most common symptoms at presentation were hypertension, blood pressure differences between limbs, and fever. The most commonly involved arteries were the abdominal aorta and renal artery. Medications included steroids, conventional and biologic DMARDs, and other immunosuppressive therapies. Half of the patients received biologic agents, of which infliximab had the highest complete remission rate (40%). Other medications resulting in complete remission were CYC (40%) and MTX (38%). Invasive procedures were required for 58% of patients. The most common complications were cardiac (50%), stroke (42%), and serious infections (33%). No patients died.
CONCLUSION
This study presents the largest series of infantile TAK. Compared with other reported series on older children, infants with TAK have more severe disease and were more likely to receive biologic agents, develop complications, and require invasive interventions.
Topics: Infant; Humans; Child; Female; Adolescent; Child, Preschool; Male; Retrospective Studies; Delayed Diagnosis; Antirheumatic Agents; Infliximab; Takayasu Arteritis; Biological Factors
PubMed: 36495199
DOI: 10.1093/rheumatology/keac691 -
Expert Review of Clinical Immunology Jul 2024Vasculitides are a heterogeneous group of disorders producing inflammation of blood vessels (e.g. arteries or veins). All major vasculitides potentially have... (Review)
Review
INTRODUCTION
Vasculitides are a heterogeneous group of disorders producing inflammation of blood vessels (e.g. arteries or veins). All major vasculitides potentially have ophthalmological symptoms and signs including visual loss. Co-morbidity, multimorbidity, polypharmacy, and geriatric syndromes all play important roles in patient outcomes for these rheumatic conditions in the elderly. This monograph reviews the NCBI PubMed database (Feb 2023) literature on the neuro-ophthalmic and geriatric considerations in vasculitis.
AREAS COVERED
Cogan Syndrome, Granulomatosis with Polyangiitis, Giant Cell Arteritis, Polyarteritis Nodosa, Takayasu Arteritis, Vasculitis epidemiology, and neuro-ophthalmological symptoms.
EXPERT OPINION
Geriatric patient care for vasculitis with neuro-ophthalmological manifestations can be complicated by the interplay of multiple co-morbidities, polypharmacy, and specific geriatric syndromes. The valuation and treatment of vasculitis and the complications associated with the disease can negatively impact patient care. Advances in noninvasive imaging and updates in diagnostic criteria have enabled increased identification of patients at earlier stages with less severe disease burden. Novel therapeutic agents can be glucocorticoid sparing and might reduce the adverse effects of chronic steroid use. Holistic care models like the 5 M geriatric care model (mind, mobility, medications, multicomplexity, and matters most) allow patients' needs to be in the forefront with biopsychosocial aspects of a patient being addressed.
Topics: Humans; Aged; Vasculitis; Comorbidity; Eye Diseases
PubMed: 38572928
DOI: 10.1080/1744666X.2024.2339893 -
The Journal of the Royal College of... Sep 2023Giant cell arteritis (GCA), otherwise known as temporal arteritis, is a form of large vessel vasculitis. Patients may present with a variety of symptoms but the most...
Giant cell arteritis (GCA), otherwise known as temporal arteritis, is a form of large vessel vasculitis. Patients may present with a variety of symptoms but the most common include headaches, jaw claudication, fatigue, night sweats and visual disturbance. Diagnosis is made using a combination of temporal artery ultrasound, clinical findings and blood tests and the diagnostic gold standard of a temporal artery biopsy and is sometimes needed for histopathological confirmation. Syphilis is a sexually transmitted disease caused by the spirochaete , which can infect the central nervous system and cause neurosyphilis, which can mimic GCA.
Topics: Humans; Giant Cell Arteritis; Temporal Arteries
PubMed: 37439491
DOI: 10.1177/14782715231184518