Vasculitis distribution and clinical characteristics in giant cell arteritis: a retrospective study using the new 2022 ACR/EULAR classification criteria.

Introduction: Giant cell arteritis (GCA) is the most common vasculitis of the elderly. In recent years, advanced imaging has to a certain extent replaced temporal artery biopsy (TAB) to aid diagnosis in many institutions and helped to identify three major phenotypes of GCA, namely, cranial GCA (c-GCA), large-vessel non-cranial GCA (LV-GCA), and a combination of these two patterns called mixed-GCA, which all show different clinical patterns. Recent 2022 American College of Rheumatology (ACR)/European League Against Rheumatism (EULAR) classification criteria respect the changing conception and clinical practice during the last two decades. In this cohort study, we present vasculitis distribution and baseline characteristics using the 2022 ACR/EULAR classification criteria as well as the EULAR core data set. Methods: In this retrospective study from Southern Norway, we identified all patients diagnosed with GCA between 2006 and 2019 in our single-center fast-track clinic (FTC). We included all patients who were examined using ultrasound (US) of cranial as well as non-cranial large vessels at diagnosis to depict vascular distribution. EULAR core data set, ACR 1990, and 2022 ACR/EULAR classification criteria were used to characterize the cohort. Results: Seventy-seven patients were diagnosed with GCA at our institution in the aforementioned period. Seventy-one patients (92.2%) were diagnosed with the help of US and included in the further analysis. The 2022 ACR/EULAR classification criteria allocated 69 patients (97.2%), while the ACR 1990 classification criteria allocated 49 patients (69.0%) in our cohort as having GCA. Mixed-GCA was the most common type in 33 patients (46.5%). Weight loss was significantly more common in patients with large-vessel non-cranial vasculitis in LV-GCA and mixed-GCA. Headache, on the other hand, was significantly more common in patients with involvement of cranial vessels. Conclusion: Mixed GCA was the most common form of GCA in our cohort. In our study, the 2022 ACR/EULAR classification criteria seem to be a more useful tool compared with the old ACR 1990 classification criteria to allocate GCA patients diagnosed and treated at our US-based FTC as having GCA.

Norwegian society of rheumatology recommendations on diagnosis and treatment of patients with giant cell arteritis.

Objective: To provide clinical guidance to Norwegian Rheumatologists and other clinicians involved in diagnosing and treating patients with giant cell arteritis (GCA). Methods: The available evidence in the field was reviewed, and the GCA working group wrote draft guidelines. These guidelines were discussed and revised according to standard procedures within the Norwegian Society of Rheumatology. The European Alliance of Associations for Rheumatology (EULAR) recommendations for imaging and treatment in large vessel vasculitis and the British Society for Rheumatology (BSR) guidelines for diagnostics and treatment in GCA informed the development of the current guidelines. Results: A total of 13 recommendations were developed. Ultrasound is recommended as the primary diagnostic test. In patients with suspected GCA, treatment with high doses of Prednisolone (40-60 mg) should be initiated immediately. For patients with refractory disease or relapse, Methotrexate (MTX) should be used as the first-line adjunctive therapy, followed by tocilizumab (TCZ). Conclusion: Norwegian recommendations for diagnostics and treatment to improve management and outcome in patients with GCA were developed.

Diagnosing giant cell arteritis: a comprehensive practical guide for the practicing rheumatologist.

GCA is the most common large vessel vasculitis in the elderly population. In recent years, advanced imaging has changed the way GCA can be diagnosed in many locations. The GCA fast-track clinic approach combined with US examination allows prompt treatment and diagnosis with high certainty. Fast-track clinics have been shown to improve prognosis while being cost effective. However, all diagnostic modalities are highly operator dependent, and in many locations expertise in advanced imaging may not be available. In this paper, we review the current evidence on GCA diagnostics and propose a simple algorithm for diagnosing GCA for use by rheumatologists not working in specialist centres.

Incidence Trends and Mortality of Giant Cell Arteritis in Southern Norway.

OBJECTIVE: Southern Norway consists of a homogeneous population of nearly 300,000 inhabitants and is an ideal epidemiologic setting. We aimed to explore potential changes in incidence of giant cell arteritis (GCA) in Southern Norway from 2000-2013, with comparisons of previous reports from the same population cohort from 1987-1994 and 1992-1996, and to investigate the mortality rates of GCA over a period of 14 years. METHODS: All patients diagnosed with GCA during January 1, 2000 to December 31, 2013 were identified through the electronic health records and biopsy findings databases at our clinic. The diagnosis of GCA and information about death was confirmed by reviewing the patients' hospital records. Inclusion criteria were: 1) fulfillment of the American College of Rheumatology 1990 criteria for GCA, or 2) histologically proven GCA, or 3) confirmed arteritis of the large or medium-sized vessels by imaging. RESULTS: A total of 206 patients were included, and 147 (72%) were females. The annual incidence rate of GCA per 100,000 inhabitants age ≥50 years was 16.8 (95% confidence interval [95% CI] 14.6-19.2), 24.5 for females (95% CI 19.2-26.5), and 10.2 for males (95% CI 7.9-13.2). Forty-six patients (22%) died (24 women, 22 men). The overall standardized mortality ratio was 1.05 (95% CI 0.77-1.38), 0.92 for females (95% CI 0.61-1.35), and 1.38 for males (95% CI 0.88-2.05). Overall survival rate was significantly higher in females compared to males (P < 0.001). CONCLUSION: GCA incidence is not increasing. We did not find excess mortality; however, males seem to have a worse survival rate compared to females.

Magnetic resonance imaging compared to ultrasonography in giant cell arteritis: a cross-sectional study.

BACKGROUND: There has been a shift in recent years to using ultrasound (US) and magnetic resonance imaging (MRI) as first-line investigations for suspected cranial large vessel vasculitis (LVV) and is a new recommendation by the EULAR 2018 guidelines for imaging in LVV. This cross-sectional study compares the performance of US and MRI and contrast-enhanced magnetic resonance angiography (MRA) for detecting vasculitis in patients with giant cell arteritis (GCA). METHODS: Patients with new-onset or already diagnosed GCA were recruited. The common temporal arteries and supra-aortic large vessels were evaluated by US and MRI/MRA. Blinded experts read the images and applied a dichotomous score (vasculitis: yes/no) in each vessel. RESULTS: Thirty-seven patients with giant cell arteritis (GCA) were recruited. Two patients were excluded. Of the remaining patients, nine had new-onset disease and 26 had established disease. Mean age was 71 years, and median C-reactive protein (CRP) was 7.5 mg/L. The median time between US and MRI was 1 day. Overall, US revealed vasculitic changes more frequently than MRI (p < 0.001). US detected vascular changes in 37% of vessels compared to 21% with MRI. Among patients with chronic disease, US detected vascular changes in 23% of vessels compared to 7% with MRI in (p < 0.001). The same was true for patients with new-onset disease. US detected vasculitic changes in 22% of vessels and MRI detected disease in 6% (p = 0.0004). Compared to contrast-enhanced MRA, US was more sensitive in detecting vasculitic changes in the large arteries, including the axillary, carotid, and subclavian arteries. CONCLUSION: US more frequently detects vasculitic changes in the large arteries compared to contrast-enhanced MRA. When evaluating the cranial vessels, US performs similarly to MRI. This data supports the recommendation that US be considered as a first-line evaluation in patients suspected to have GCA.

The fast-track ultrasound clinic for early diagnosis of giant cell arteritis significantly reduces permanent visual impairment: towards a more effective strategy to improve clinical outcome in giant cell arteritis?

OBJECTIVE: Permanent visual impairment has been reported to occur in up to 19% of GCA patients. The aim of this study was to examine whether implementation of a fast-track approach could reduce the rate of permanent visual impairment and inpatient days of care in GCA patients. METHODS: A fast-track outpatient GCA clinic (FTC) was implemented in the Department of Rheumatology, Hospital of Southern Norway Trust Kristiansand, Norway in 2012. The patients included in this study were subsequently recruited between March 2010 and October 2014. Routine clinical and laboratory data and number of inpatient days of care were collected. RESULTS: During the observation period, 75 patients were diagnosed with GCA. Among the 75 GCA patients, 32 were evaluated conventionally and 43 in the FTC. In the conventionally approached group, six patients suffered from permanent visual impairment, while in the FTC group only one patient suffered from permanent visual impairment. The relative risk of permanent visual impairment in the GCA patients examined in the FTC was 88% lower compared with the conventionally evaluated group (relative risk 0.12, 95% CI: 0.01, 0.97, P = 0.01). The mean difference in inpatient days of care between patients evaluated conventionally and patients evaluated in the FTC was 3 days (3.6 vs 0.6 days, P < 0.0005). CONCLUSION: The implementation of the FTC in GCA care appears to significantly reduce the risk of permanent visual impairment and is more cost effective by reducing the need for inpatient care.

Diagnostic value of color Doppler ultrasonography of temporal arteries and large vessels in giant cell arteritis: a consecutive case series.

OBJECTIVE: Color Doppler ultrasonography (CDUS) can detect inflammation in the vessel wall. No studies have evaluated the examination of the common carotid artery by CDUS in the diagnostics of giant cell arteritis (GCA). Our aim was to evaluate the combination of CDUS examination of the temporal, axillary, and common carotid arteries in the diagnosis of GCA. METHODS: Patients ages ≥50 years who were referred to our department between April 2010 and October 2012 and suspected to have GCA were consecutively examined. A positive clinical evaluation for GCA 6 months after the first evaluation by 3 rheumatologists was considered as the gold diagnostic standard. All patients underwent CDUS of the temporal, axillary, and common carotid arteries. A biopsy of the temporal artery was performed for most patients. RESULTS: A total of 88 patients were assessed. Forty-six patients were diagnosed to have GCA by the defined gold standard. Forty-eight patients had a positive CDUS of the temporal artery. Forty-six patients diagnosed with GCA had a positive CDUS of the temporal, common carotid, and axillary arteries (100% sensitivity) and 4 patients had a positive CDUS without having GCA (91% specificity). Among the 39 GCA patients that underwent a biopsy, vasculitis was observed in 26 patients (66%), yielding a sensitivity of 67% and a specificity of 95%. CONCLUSION: CDUS of the common carotid, axillary, and temporal arteries had an excellent sensitivity and high specificity to diagnose GCA. CDUS has the potential to replace biopsy in ordinary clinical care without compromising on sensitivity and specificity.

Leflunomide as a corticosteroid-sparing agent in giant cell arteritis and polymyalgia rheumatica: a case series.

OBJECTIVES: Giant cell arteritis (GCA) and polymyalgia rheumatica (PMR) affect individuals older than 50 years of age and corticosteroids are the mainstay of treatment. The aim of our study was to explore the role of leflunomide as a corticosteroid-sparing agent in GCA and PMR patients. METHODS: Patients with difficult-to-treat GCA and PMR were retrospectively identified in the period from 2010 to 2013. The doses of corticosteroids and CRP values were noted before, after three months, and at the end of the treatment with leflunomide (for patients continuing treatment, censoring date was January 1, 2013). RESULTS: Twenty-three patients were identified (12 with PMR and 11 with GCA). A reduction of 6 mg/dL (CI 95% -10.9-34.2, P = 0.05) in CRP and 3.7 mg (CI 95% 0.5-7.0, P = 0.03) in prednisolone dose was observed in the PMR group. In GCA patients, the reduction was 12.4 mg/dL (CI 95% 0.7-25.5, P = 0.06) in CRP and 6.6 mg (CI 95% 2.8-10.3, P < 0.01) in prednisolone dose. CONCLUSION: Leflunomide seems to be effective as a corticosteroid-sparing agent in patients with difficult-to-treat GCA and PMR. Randomized controlled trials are warranted in order to confirm the usefulness of leflunomide in the therapy of GCA/PMR.

Severe deterioration of newly diagnosed Takayasu arteritis in a patient re-treated with interferon beta-1α for concomitant longstanding multiple sclerosis.

A 52-year-old woman with Takayasu arteritis and a known history of multiple sclerosis had been treated with subcutaneous interferon (IFN) beta-1α. After the re-introduction of the IFN beta-1α, the patient had a gradual worsening of the arteritis, with claudication symptoms in the left arm and increased inflammation markers. An evaluation using Doppler ultrasound of the supra-aortic vessels revealed severe stenosis of the left axillary artery. The IFN beta-1α was withdrawn, with prompt clinical and laboratory improvement of the vasculitis.

Causes of death in polymyalgia rheumatica. A prospective longitudinal study of 315 cases and matched population controls.

OBJECTIVE: To determine causes of death in patients with pure polymyalgia rheumatica (PMR) compared to matched population controls. METHODS: In a population based study from 1987-1997, 315 patients were diagnosed with PMR. The patients were each randomly assigned four population controls, totally 1,260 controls. The date and causes of death were identified from the data files at Statistics Norway up to the end of 1997. RESULTS: A total of 65 cases (20.6%) with PMR died compared to 338 (26.8%) among the controls (mortality rate ratio (MRR) = 0.73, 95% CI 0.56-0.97, p = 0.03). No statistically significant difference was found between patients and controls with regard to mortality from coronary heart disease or stroke (MRR=0.78, 95% CI 0.52-1.18), cancer (MRR = 0.59, 95% CI 0.30-1.17), and other causes (MRR=0.75, 95% CI 0.48-1.17). CONCLUSION: The increased survival found in patients with PMR could not be explained by reduction in any particular cause of death.

No increased frequency of malignant neoplasms in polymyalgia rheumatica and temporal arteritis. A prospective longitudinal study of 398 cases and matched population controls.

OBJECTIVE: To determine the prevalence and incidence of cancer in patients with polymyalgia rheumatica (PMR) and temporal arteritis (TA) compared to matched population controls. METHODS: In a population based study 1987-97, 398 patients were diagnosed with PMR or TA. Each patient was randomly assigned 4 age and sex matched controls from the same county, totaling 1592 controls. All patients and controls were cross-checked with data files at the Cancer Registry of Norway, for cancers registered up to the end of 1998. RESULTS: Prior to inclusion, cancer was diagnosed in 32 patients with PMR or TA (8.0%) and 153 controls (9.6%) (OR 0.82, 95% CI 0.55-1.22, p = 0.3). After inclusion, malignant neoplasms were discovered in 34 patients with PMR or TA (9.3%) compared to 143 controls (10.8%) (relative risk 0.86, 95% CI 0.59-1.26, p = 0.4). Thus there was no difference between patients with PMR or TA and their controls regarding prevalence or incidence of cancer. The interval between inclusion and the time of diagnosis of malignant neoplasm did not differ between patients and controls. No significant difference in types or localization of malignant neoplasms was found in patients compared to controls. CONCLUSION: No differences were found in frequencies or types of malignant neoplasms between patients with PMR or TA and population controls. Neither PMR nor TA as defined by present diagnostic criteria appears associated with cancer.

[Control of patients in an outpatient clinic of rheumatology]. / Kontroll av pasienter ved en revmatologisk poliklinikk.

BACKGROUND: A high number of patients attending outpatient clinics of rheumatology are control cases. This study aimed at investigating the causes of such controls and how necessary they are. MATERIAL AND METHODS: 400 consecutive control patients selected from a local hospital were included. RESULTS: Inflammatory rheumatic diseases constituted 89% of the controls; rheumatoid arthritis was the diagnosis in 28% of cases. Routine control of patients with chronic disease was the main cause of control (41%). 15% of controls were regarded as partly or completely unnecessary; 95% of these were not repeated. INTERPRETATION: The reasons for controlling patients in rheumatology should be better defined. Regular evaluation could result in improved service to outpatients.