Primary Systemic Vasculitis Research Articles

Giant Cell Arteritis: A review of current knowledge and new biological treatment options

Introduction and objective Giant cell arteritis (GCA) is the most common primary systemic vasculitis in Europe. It mainly affects individuals over the age of fifty, its incidence increases with age. The most common clinical manifestation is bilateral headache accompanied by systemic symptoms. The disease may be associated with serious complications, such as permanent vision loss, stroke or rupture of aortic aneurysm. The aim of this study is to discuss the current knowledge about giant cell arteritis and new possibilities of biological treatment. Review methodsTo prepare this review, PubMed and Google Scholar databases were used, searching the following terms: “giant cell arteritis,” “temporal arteritis,” “giant cell arteritis biological treatment,” “giant cell arteritis biological therapy,” “giant cell arteritis treatment,” “giant cell arteritis epidemiology.” The search results were limited to publications from 2007 to 2024, as well as key studies from earlier years, including original papers and randomized, double-blind, placebo-controlled studies. Brief description of the state of knowledgeThe pathogenesis of GCA remains unclear. Since inflammatory factors appear to play an important role, new therapeutic strategies are aimed in direction to interfere these pathways. Setting a diagnosis of GCA requires imaging studies or a temporal artery biopsy. The results of current treatment with high doses of glucocorticoids are unsatisfying, which has led to ongoing research into new therapeutic options. SummaryEarly diagnosis and initiation of treatment are crucial for preventing occurrence of complications. Although the current treatment regimen is effective, it leads to many side effects and does not prevent relapses. This highlights the need to better understand the pathomechanism of the disease to develop more precise therapeutic strategies, including biological treatment.

Mechanisms and Screening for Atherosclerosis in Adults With Vasculitis.

Vascular inflammation is a hallmark of both primary systemic vasculitis and atherosclerosis. As such, cardiovascular events are common in patients with vasculitis and likely due to both direct vascular inflammation and accelerated atherosclerosis. Direct cardiac involvement is possible in all vasculitides, though more commonly described in Takayasu arteritis, polyarteritis nodosa, and eosinophilic granulomatosis with polyangiitis. Accelerated atherosclerosis has been described in Takayasu arteritis and antineutrophil cytoplasmic antibody-associated vasculitis, though there remains a paucity of data in other forms of vasculitis. Multiple screening and management approaches for cardiovascular risk in people with vasculitis have been proposed, though evidence-based guidelines are lacking. In this review, we discuss the latest evidence in epidemiology, mechanisms, and screening for atherosclerosis in patients with primary systemic vasculitides.

The value of ultrasound for monitoring disease activity in giant cell arteritis

Giant cell arteritis (GCA) is the most common primary systemic vasculitis in the elderly. Although the diagnosis of GCA has improved, monitoring its disease activity remains challenging due to the lack of validated tools and biomarkers. The current reliance on assessing symptoms, physical signs, and inflammatory markers during disease follow-up presents limitations, notably the nonspecific nature of GCA-related symptoms and the suppressive impact of IL-6 inhibitors on inflammatory markers. Therefore, recent attention has shifted toward acknowledging imaging as a monitoring tool, particularly ultrasound, given its widespread accessibility, cost-effectiveness, and well-established role in GCA diagnosis. Research on this topic has found that ultrasound characteristics, including the number of affected arterial segments and halo size, are associated with laboratory markers and treatment response, underscoring the ultrasound’s potential as a monitoring tool for GCA. It has also been demonstrated that ultrasound abnormalities progress differently throughout the disease course, depending on the type of arterial involvement, with vessel wall changes in the axillary arteries resolving more slowly than those in the temporal arteries. Nevertheless, there are still no studies comparing the added value of regular ultrasounds for monitoring disease activity to clinical and laboratory monitoring alone; hence, this imaging modality is not yet recommended for patients with GCA in clinical and biochemical remission. This narrative review aims to synthesize the main research findings of key studies addressing the role of ultrasound for monitoring disease activity in GCA, with a focus on the pattern of arterial involvement. It highlights the potential of ultrasound, particularly halo sign assessment, for evaluating disease progression but notes that further validation and standardization of protocols are needed to improve accuracy and enable routine use.

The primary systemic vasculitis associated optic neuritis: a retrospective analysis in a single center over 10 years

ObjectivesTo investigate the clinical and image characteristics of primary systemic vasculitis-associated optic neuritis patients.MethodsThis is a retrospective study. The patients clinically diagnosed with primary system vasculitis-induced optic neuritis were recruited from March 2013 to December 2023. All cases received orbital magnetic resonance imaging scans were analyzed. The ocular findings, systemic manifestations, laboratory data and prognosis were reviewed retrospectively. In addition, the related literature was reviewed.ResultsFourteen patients (21 eyes), including 10 men and 4 women, were enrolled in this study. The ages ranged from 30 to 86 years in this cohort. Orbits MRI detects the enlargement and/or enhancement of the optic nerve. Cases 1–5 reported a confirmed diagnosis of Takayasu’s arteritis, and cases 6–8 had giant cell arteritis. Cases 9–13 were antineutrophil cytoplasmic antibody-associated vasculitis. Case 14 was Cogan’s syndrome. Mult organs and tissues, such as the kidneys, heart, paranasal sinuses, meninges, and respiratory system, were involved. In all of the 14 involved patients, the disease onset was either during the fall or winter season. There were no or only slight improvements in visual activity after conventional therapies.ConclusionsThe autoantibodies’ attack on the optic nerve, ischemic damage, or destruction of the blood–brain barrier may be the potential pathogenesis of vasculitis-associated optic neuritis. Even with prompt and aggressive clinical interventions, the prognosis remains unsatisfactory.

Peripheral neuroimmunological diseases - Neuropathological insights and clinical perspectives

This article deals with peripheral neuroimmunological diseases and briefly outlines the currently most important aspects and treatment developments. Idiopathic inflammatory myopathies have different mechanisms of development, manifestations and prognoses. New classification systems and more specific treatment concepts have been developed. The IIMs include different subgroups. These entities can have specific autoantibodies. Diagnostically, amuscle biopsy is generally desirable for aprecise diagnosis and is essential in unclear cases. Primary systemic vasculitides can be divided into different groups based on the predominant pattern of involvement, while secondary vasculitides and single organ vasculitides are also differentiated. Vasculitic myopathy cannot be equated with myositis and areliable distinction is currently only possible by a muscle biopsy. Treatment concepts should be developed on an interdisciplinary basis. Chronic inflammatory demyelinating polyneuropathy is the most frequent immune-mediated neuropathy and is characterized by apredominant demyelination of the motor and sensory nerves. The disease course runs in phases or is progressive and leads to significant disability and reduction in quality of life, despite current standard treatment. Novel treatment approaches are currently undergoing clinical trials. Myasthenia gravis, with the leading symptom of exercise-induced muscle weakness, is caused by autoantibodies against structures of the neuromuscular endplate. Autoantibody testing is the most important pillar in the diagnosis and is now also increasingly guiding treatment decisions. Overall, peripheral neuroimmunological diseases represent aheterogeneous group. Increasing knowledge of the pathophysiology is the key to numerous developments in diagnostics and treatment, which could lead to far-reaching practical changes in the future.

Imaging findings in Giant Cell Arteritis: Don't Turn A Blind Eye To The Obvious!

Giant cell arteritis (GCA) is the most common primary large vessel systemic vasculitis in the western world. Even though the involvement of scalp and intracranial vessels has received much attention in the neuroradiology literature, GCA, being a systemic vasculitis can involve multiple other larger vessels including aorta and its major head and neck branches. Herein, the authors present a pictorial review of the various cranial, extracranial and orbital manifestations of GCA. An increased awareness of this entity may help with timely and accurate diagnosis, helping expedite therapy and preventing serious complications.ABBREVIATIONS: ACR= American College of Rheumatology, AION= Anterior Ischemic Optic Neuropathy, EULAR= European League Against Rheumatism, GCA= Giant Cell Arteritis, LV-GCA= Large vessel GCA, PMR= Polymyalgia Rheumatica, US= Ultrasound, VWI= Vessel Wall Imaging.

Current perspective on infections and mitigation strategies in primary systemic vasculitis.

The purpose of this review is to summarize and evaluate most recent evidence on the epidemiology of infections and associated risk factors in patients with primary systemic vasculitides (PSV), as well as discuss mitigation strategies including the risk of antibiotic prophylaxis. Infections remain one of the leading causes of mortality in patients with PSV, with rates of severe infection ranging from 16 to 40% in different cohorts. Older age, frailty, renal and pulmonary involvement, and higher burden of comorbidities have been recognized as important patient-associated risk factors. Treatments including higher cumulative doses of glucocorticoids are associated with an increased risk of infections, and recent studies show the potential benefit of interventions such as reduced-dose glucocorticoid regimens. Existing mitigation strategies include screening, vaccination, and infection prophylaxis. The latter remains particularly important for Pneumocystis jirovecii pneumonia; however, the benefit-risk ratio seems to be less clear outside of induction phase (i.e., high dose of glucocorticoids) and optimal treatment duration remains less clear. Patients with PSV are at increased risk of infections, due to disease itself, comorbidities, and treatment side effects. Awareness of the timing and types of infection, as well as mitigation strategies are imperative to ensure treatment success and survival for patients.

Association Between Wearable Device Use and Quality of Life in Patients With Idiopathic Inflammatory Myopathies and Primary Systemic Vasculitis.

Background Despite the increasing use of wearable devices worldwide, concise data on these instruments in patients with systemic autoimmune rheumatic diseases, including idiopathic inflammatory myopathies (IIM) and primary systemic vasculitis (PSV), are lacking. Objectives The aim of this study is to investigate the knowledge and use of wearable devices and to assess their impact on the general quality of life of patients with IIM and PSV. Moreover, we compared these characteristics between patients with IIM and PSV users and non-users of wearable devices. Methods This single-center, cross-sectional study was conducted between January 2023 and June 2023. We included adult patients with IIM and PSV and a control group (CTR) and evaluated their use of cell phones and wearables, level of physical activity, and quality of life. Results A total of 132 patients with IIM, 82 with PSV, and 178 in the CTR were evaluated. Overall, 169 patients and 144 in the CTR were aware of wearable devices, of whom 50 (29.6%) and 47 (32.6%), respectively, had already used this technology. In addition, the IPAQ-Mets and EQ-5D scores were lower in the IIM and PSV groups than in the CTR, and the fatigue severity scale (FSS) scores were higher in the IIM and PSV groups than in the CTR. Patients who used the devices showed FSS scores of 29 (18-40) points, with higher levels of IPAQ-Mets among device users, indicating greater physical activity than among nonusers. Conclusion Based on the results, the use of wearable devices is associated with better fatigue and IPAQ scores. Possibly, the use of such devices can have an impact on better lifestyle habits among these patients.

Anti-LAMP-2 Antibody Seropositivity in Children with Primary Systemic Vasculitis Affecting Medium- and Large-Sized Vessels.

Chronic primary systemic vasculitis (PSV) comprises a group of heterogeneous diseases that are broadly classified by affected blood vessel size, clinical traits and the presence (or absence) of anti-neutrophil cytoplasmic antibodies (ANCA) against proteinase 3 (PR3) and myeloperoxidase (MPO). In small vessel vasculitis (SVV), ANCA are not present in all patients, and they are rarely detected in patients with vasculitis involving medium (MVV) and large (LVV) blood vessels. Some studies have demonstrated that lysosome-associated membrane protein-2 (LAMP-2/CD107b) is a target of ANCA in SVV, but its presence and prognostic value in childhood MVV and LVV is not known. This study utilized retrospective sera and clinical data obtained from 90 children and adolescents with chronic PSV affecting small (SVV, n = 53), medium (MVV, n = 16), and large (LVV, n = 21) blood vessels. LAMP-2-ANCA were measured in time-of-diagnosis sera using a custom electrochemiluminescence assay. The threshold for seropositivity was established in a comparator cohort of patients with systemic autoinflammatory disease. The proportion of LAMP-2-ANCA-seropositive individuals and sera concentrations of LAMP-2-ANCA were assessed for associations with overall and organ-specific disease activity at diagnosis and one-year follow up. This study demonstrated a greater time-of-diagnosis prevalence and sera concentration of LAMP-2-ANCA in MVV (52.9% seropositive) and LVV (76.2%) compared to SVV (45.3%). Further, LAMP-2-ANCA-seropositive individuals had significantly lower overall, but not organ-specific, disease activity at diagnosis. This did not, however, result in a greater reduction in disease activity or the likelihood of achieving inactive disease one-year after diagnosis. The results of this study demonstrate particularly high prevalence and concentration of LAMP-2-ANCA in chronic PSV that affects large blood vessels and is seronegative for traditional ANCA. Our findings invite reconsideration of roles for autoantigens other than MPO and PR3 in pediatric vasculitis, particularly in medium- and large-sized blood vessels.

Stroke frequency, associated factors, and clinical features in primary systemic vasculitis: a multicentric observational study.

The cerebral vessels may be affected in primary systemic vasculitis (PSV), but little is known about cerebrovascular events (CVEs) in this population. This study aimed to determine the frequency of CVEs at the time of diagnosis of PSV, to identify factors associated with CVEs in PSV, and to explore features and outcomes of stroke in patients with PSV. Data from adults newly diagnosed with PSV within the Diagnostic and Classification Criteria in VASculitis (DCVAS) study were analysed. Demographics, risk factors for vascular disease, and clinical features were compared between patients with PSV with and without CVE. Stroke subtypes and cumulative incidence of recurrent CVE during a prospective 6-month follow-up were also assessed. The analysis included 4828 PSV patients, and a CVE was reported in 169 (3.50%, 95% CI 3.00-4.06): 102 (2.13% 95% CI 1.73-2.56) with stroke and 81 (1.68% 95% CI 1.33-2.08) with transient ischemic attack (TIA). The frequency of CVE was highest in Behçet's disease (9.5%, 95% CI 5.79-14.37), polyarteritis nodosa (6.2%, 95% CI 3.25-10.61), and Takayasu's arteritis (6.0%, 95% CI 4.30-8.19), and lowest in microscopic polyangiitis (2.2%, 95% CI 1.09-3.86), granulomatosis with polyangiitis (2.0%, 95% CI 1.20-3.01), cryoglobulinaemic vasculitis (1.9%, 95% CI 0.05-9.89), and IgA-vasculitis (Henoch-Schönlein) (0.4%, 95% CI 0.01-2.05). PSV patients had a 11.9% cumulative incidence of recurrent CVE during a 6-month follow-up period. CVEs affect a significant proportion of patients at time of PSV diagnosis, and the frequency varies widely among different vasculitis, being higher in Behçet's. Overall, CVE in PSV is not explained by traditional vascular risk factors and has a high risk of CVE recurrence.

Maternal and fetal outcomes of pregnancy in women with primary systemic vasculitis: A single-center cohort study of 20 patients and 30 pregnancies

ObjectivesTo analyze pregnancy outcomes of patients with primary systemic vasculitis followed in a third-level referral center. MethodsRetrospective cohort study of all pregnant women with systemic vasculitis followed between 2009 and 2022 at the High-Risk Pregnancy Clinic of the Department of Systemic Autoimmune Diseases of the Hospital Clínic, Barcelona. ResultsTwenty women with primary vasculitis were identified, with a total of 30 pregnancies. Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (n = 7) and Behçet disease (n = 4) were the most frequent types of vasculitis. All women had the diagnosis of vasculitis before pregnancy, with a median time between disease diagnosis and pregnancy of 5.8 years (range: 2 months-29 years). Most were in remission at conception (76.7 %). During pregnancy, a vasculitis flare occurred in 4 (13.3 %) patients (one each with Takayasu arteritis, eosinophilic granulomatosis with polyangiitis [EGPA], IgA vasculitis [IgAV], and Behçet disease [BD]). Four (16.7 %) of the successful pregnancies had post-partum relapses (one each with EGPA, granulomatosis with polyangiitis, IgAV, and BD). Eighty percent of pregnancies resulted in live babies. In four cases (13.3 %), medical termination of pregnancy was decided, considering the mother or baby health risk. There were two spontaneous miscarriages, and no stillbirths or neonatal deaths. Preeclampsia was the most frequent maternal complication (25 %). Newborns were preterm in 24 % and low birthweight in 20 % of cases. No maternal deaths occurred. ConclusionsThis cohort study shows that vasculitis relapses during pregnancy and post-partum, together with other pregnancy complications, occur in a considerable number of patients with systemic vasculitides, although a final good pregnancy outcome can be expected in most cases. These findings emphasize the convenience of managing these special situations in expert reference centers.

An Observational Cohort Study to Estimate the Durability of Humoral Immune Responses to Vaccination with ChAdOx1 nCoV-19 and the BBV152 in Adult Patients with Autoimmune Rheumatic Diseases (AIRD)

Background The durability of immune responses after vaccination against SARS CoV-2 in patients with autoimmune rheumatic diseases (AIRD) is not well studied. Objective We aimed to determine the longevity of humoral immune response to homologous vaccination with 2 doses of ChAdOx1 nCoV-19 and BBV152 in adult patients with AIRD. Methods In this prospective observational study, patients aged 18 to 60 years, diagnosed with SLE, primary systemic vasculitis and inflammatory arthritis (IA) who were scheduled to or had received vaccination with either ChAdOx1 nCoV-19 or BBV152 were recruited between June 2021 to March 2023. IgG antibodies against SARS CoV-2 spike protein (anti-S) were estimated in serum or plasma of patients prior to vaccination if feasible (T0), 15 to 28 days (T1), 75-100 days (T2), 170-190 days (T3), 240-290 days (T4), 340-370 days (T5) after receiving Vx2 by Diasorin chemiluminescence assay. The association of antibody titers with baseline parameters is estimated using chi-square or Mann Whitney tests using SPSS 16. Results Overall, 118 patients (93 females; age: 37.1, IQR: 28- 45 years; disease duration: 60 ((IQR) 29-103) months) with AIRD (SLE:48(40.6%), IA:53(44.9%), systemic vasculitis:7(5.9%) and spondyloarthropathy: 9(7.6%)) were recruited. Baseline demographic data has been depicted in (Table 1). The median antibody titer did not differ significantly between the two vaccines at T1 while the titers at T2, T3 and T4 were significantly lower for those vaccinated with BBV 152 (Table 2). The steroid dose prior to T1 and T2, intake of DMARDs in peri-vaccination period, type of AIRD (IA vs CTD) was not associated with significant difference in antibody titers at T1 and T2. Conclusion The anti-S humoral immune response persisted beyond 1 year for most of our patients with AIRD. After 3 months of vaccination, these responses were higher for ChAdOx1 nCoV-19 as compared with BBV152 in our patients with AIRD.

E37 Clinical and demographic criteria of Juvenile primary systemic vasculitis in Libyan Children

Abstract Introduction Primary vasculitis syndromes are rare in childhood. The clinical manifestations depend on the vessels involved and can lead to significant mortality and morbidity. Aim To study demographic and clinical characteristics of patients with diagnosed with vasculitis other than Kawazaki disease and Henoch-Schonlein purpora (HSP) and to report the outcome of the patients. Materials and methods This is a case series conducted by reviewing the files of patients diagnosed with vasculitis and followed up at the pediatric rheumatology unit of the Tripoli Children Hospital, Libya from year 2000–2022. Data regarding patients’ demography, time of diagnosis, clinical features, laboratory results, treatment and complications were retrieved. Results A total of 37 cases included in the study. 17 (45.9%) were males and 20 (54%) were females followed up for a mean period of 4.48 ± 4.21. Mean age at diagnosis was 8.39 ± 3.84 years. Regarding the diagnosis, there were 3 (8.1%) patients with Takayasu arteritis, 5 (13.5%) patients with polyarteritis nodosa, 2 (5.4%) patients with cutaneous polyarthritis nodosa, 1 (2.7%) patients with granulomatosis with polyahgitis, 1 (2.7%) patient with microscopic polyangiitis, 3 (8.1%) patients with Chrug-Strauss disease, 16 (43.2%) patients with Behcet disease, 1 (2.7%) patients with cogan syndrome, 3(8.1%) patients with urticarial vasculitis, 1 (2.7%) patient with hypocomplementaemic vasculitis, and 1 (2.7%) patient with DADA2. Mean period between the start of symptoms and diagnosis was 1.4 ± 2.8 years. In 6 (16.2%) of the patients there was family history of vasculitis and in 11 (29.7%) the parents are consanguineous. Angiography revealed abnormalities in 5 (13.5%) of the patients. Skin biopsy was done in 4 (10.8%) patients [2 patients with polyarthritis nodosa and 2 patients with urticarial vasculitis] and renal biopsy was done in 1 (2.7%) patient. Steroid was used in 24 (64.9%), Azathioprine in 4 (10.8%) patients. Cellcept was used in 10 (27%) of the patients. Ciclosporin was used 1(2.7%) patient, cyclophosphamide in 3 (8.1%) patients and Immunoglobulin in 6 (16.2%) of patients. Biologics were used in six patients, adalimumab in one patient which was discontinued because of recurrent abscess. In the two patients who used infliximab, treatment was effective. Three patients were treated with tocilizumab, it was ineffective in two, therefore discontinued. However, tocilizumab was effective in one patient with Takayasu arteritis. Complications developed in 23 (62.2%). Muscle atrophy in 1, deforming arthritis in 1, osteoporosis in 1, mouth ulcers in 2, hypertension in 2, hearing loss in 1, chronic sinusitis in 1, cardiovascular accident in 1, cataract in 1, visual impairment in 3, proteinuria in 2, pulmonary fibrosis in 1, chronic asthma in 3, impaired renal function in 1. Conclusion This is the first report of pediatric vasculitis cases in Libya. It showed a heterogeneous spectrum of vasculitis with high rate of consanguinity and low rate of reported family history of vasculitis. Hopefully this report will increase awareness among clinicians of vasculitis and more cases will be diagnosed.

Ethnic association in primary systemic vasculitis: A systematic review

Background: Literature described wide disparities in incidence and prevalence between different types of vasculitis. There were no comprehensive studies on ethnic or racial associations in all types of primary systemic vasculitis (PSV) in any published article, until this review commenced in 2020. The purpose of the review is to synthesize the evidence regarding the relation of ethnicity and the incidence and/or prevalence of different types of PSV. Methods: A total of 52 selected articles which include Clinical trials, cohorts, cross-sectional studies, case series, and case studies and have been published within the last 10 years in the human population, were reviewed by searching The Medline, PubMed, and Google Scholars databases using predefined keywords. The PRISMA diagrams were followed to identify relevant articles. The methodological qualities of the studies were assessed using the EPHPP tool. Finally, a summary of the evidence on the association between ethnic origin and PSV was painstakingly compiled. Results: The connection between ethnicity and different types of PSV has been found to be significantly diverse in this research such as vasculitis is more common in Asians and Scandinavians, Kawasaki disease and periarteritis nodules are more prevalent in Japanese and Alaska-natives, ANCA-associated vasculitis is more frequent in Caucasians, whereas Henoch-Schonlein purpura and Cogan syndrome more usual in Caucasians and Asians. Furthermore, Behçet's disease more commonly occurs on the "Silk Road", especially in Turkey. Conclusion: Genetic susceptibility and environmental elements could be the contributing factors to the global variation in the incidence and prevalence of primary systemic vasculitis.

OA33 The incidence of large vessel vasculitis in Norfolk

Abstract Background/Aims There are no data on the collective incidence of the large vessel vasculitides. The incidence of GCA and Takayasu arteritis in the UK has been based on clinical coding in routine administrative datasets. There are no data on the incidence of these diseases based on clinically verfied diagnoses. We studied the incidence of the large vessel vasculitides in a stable population with a predominant Northern European ancestry. Methods Individuals attending a secondary care hospital with a clinically verified diagnosis of primary systemic vasculitis made between 2011-2020, who lived within the NR postcode districts of Norfolk county were included if they met classification criteria for GCA or Takayasu arteritis, or had definite tissue or imaging evidence of large vessel vasculitis. Population above the age of 18 as in the 2011 census, available from the office of national statistics, was accepted as the denominator. If classification criteria for both GCA and TAK were met, physician judgement was accepted as final diagnosis. Results 272 adults were diagnosed with large vessel vasculitis out of a population of 454,316. The annual incidence of large vessel vasculitis in Norfolk is 59.9/million in the adult population. The annual incidence of GCA is 9.9/100,000 over the age of 50 using the ACR 1990 criteria and 10.6/100,000 using the ACR/EULAR 2022 criteria. The rise in the incidence from 2017 onwards coincides with the establishment of a fast-track pathway (Table 1). The dip in the incidence in 2020 coincides with suspension of services during the SARS-CoV-2 pandemic. The annual incidence peaks at 168.5/100,000 in the 9th decade and is commoner in females (12.3/100,000 vs 7.3/100,000). The annual incidence of Takayasu arteritis is 3.3/million in the adult population using the ACR 1990 criteria and 1.1/million using the ACR/EULAR 2022 criteria. Conclusion This is the first study that reports the incidence of all objectively diagnosed large vessel vasculitis in a stable population in Norfolk county. The incidence of GCA rose with the establishment of a fast-track pathway and its peak may have been affected by the SARS-CoV-2 pandemic. GCA is commoner in females and peaks in the 8th and 9th decades. Disclosure C.B. Mukhtyar: None. C. Beadsmoore: None. F. Coath: None. G. Ducker: None. K. Sisson: None. R.A. Watts: None.

Neuropathies périphériques au cours des maladies de système : partie II (vascularites)

Primary systemic vasculitides, mainly of the small and medium-sized vessels, are frequently associated with peripheral neuropathies. When the disease is already known, the appearance of a neuropathy should suggest a specific injury, especially when associated with other systemic manifestations. Conversely, when neuropathy is inaugural, close collaboration between neurologists and internists is necessary to reach a diagnosis. A standardized electro-clinical investigation specifying the topography, the evolution and the mechanism of the nerve damage enables the positive diagnosis of the neuropathy. Several elements orient the etiological diagnosis and allow to eliminate the main differential diagnosis: non systemic vasculitic neuropathy. The existence of associated systemic manifestations (glomerular or vascular nephropathy, interstitial lung disease, intra-alveolar hemorrhage, ENT involvement…), biological markers (ANCA, cryoglobulinemia, rheumatoid factor), and invasive examinations allowing histological analysis (neuromuscular biopsy) are all useful tools for.

Management of Patients Affected by Giant Cell Arteritis during the COVID-19 Pandemic: Telemedicine Protocol TELEMACOV.

Giant cell arteritis (GCA) is the most common primary systemic vasculitis in western countries, prevalently affecting elderly people. Both early diagnosis and regular monitoring are necessary for the correct management of GCA. Following the outbreak of the COVID-19 pandemic, government decisions aiming at reducing the contagion led to reductions in health activities, limiting them to urgent cases. At the same time, remote monitoring activities have been implemented through telephone contacts or video calls carried out by specialists. In line with these deep changes affecting the worldwide healthcare system and in consideration of the high risk of GCA morbidity, we activated the TELEMACOV protocol (TELEmedicine and Management of the patient affected by GCA during the COVID-19 pandemic) in order to remotely monitor patients affected by GCA. The aim of this study was to evaluate the effectiveness of telemedicine in the follow-up of patients already diagnosed with GCA. This was a monocenter observational study. Patients with a previous diagnosis of GCA admitted to the Rheumatology Unit of the University Hospital "Città della Salute e della Scienza" in Turin were monitored every 6-7 weeks by means of video/phone calls from 9 March to 9 June 2020. All patients were asked questions concerning the onset of new symptoms or their recurrence, exams carried out, changes in current therapy, and satisfaction with video/phone calls. We performed 74 remote monitoring visits in 37 GCA patients. Patients were mostly women (77.8%) and had a mean age of 71.85 ± 9.25 years old. The mean disease duration was 5.3 ± 2.3 months. A total of 19 patients received oral glucocorticoids (GC) alone at the time of diagnosis with a daily dose of 0.8-1 mg/kg (52.7 ± 18.3 mg) of prednisone, while 18 patients were treated with a combination of oral steroids (at the time of diagnosis, the prednisone mean dose was 51.7 ± 18.8 mg) and subcutaneous injections of tocilizumab (TCZ). During the follow-up, patients additionally treated with TCZ reduced their GC dose more than patients treated with GC alone (p = 0.03). Only one patient, who was treated with GC alone, had a cranial flare and needed to increase the dosage of GC, which led to rapid improvement. Furthermore, all patients proved very adherent to the therapies (assessed by Medication Adherence Rating Scale (MARS)) and considered this type of monitoring very satisfactory according to a Likert scale (mean score 4.4 ± 0.2 on a 1-5 range). Our study shows that telemedicine can be safely and effectively used in patients with GCA under control as a possible alternative, at least for a limited period of time, to traditional visits.

Ethnicity association in primary systemic vasculitis : A systematic search and review

Literature described wide disparities in incidence and prevalence between different types of vasculitis. There were no comprehensive studies on ethnic or racial associations in all types of primary systemic vasculitis (PSV) in any published article until this review commenced in 2020. The review aims to synthesize the evidence regarding the relation between ethnicity and the incidence and/or prevalence of different types of PSV. A total of 52 selected articles which include clinical trials, cohorts, cross-sectional studies, case series, and case studies and have been published within the last 10 years in the human population, were reviewed by searching Medline, PubMed, and Google Scholars databases using predefined keywords. The PRISMA diagrams were followed to identify relevant articles. The methodological qualities of the studies were assessed using the EPHPP tool. Finally, a summary of the evidence on the association between ethnic origin and PSV was painstakingly compiled. The connection between ethnicity and different types of PSV has been found to be significantly diverse in this researchas vasculitis is more common in Asians and Scandinavians, Kawasaki disease and periarteritis nodules are more prevalent in Japanese and Alaskanatives,ANCAassociated vasculitis is more frequent in Caucasians, whereas Henoch-Schonleinpurpura and Cogan syndrome more usual in Caucasians and Asians. Furthermore, Behçet's disease more commonly occurs on the "Silk Road," especially in Turkey. Genetic susceptibility and environmental elements could contribute to the global variation in the incidence and prevalence of primary systemic vasculitis.

Accuracy of Doppler ultrasound in the diagnosis of giant cell arteritis: a systematic review and meta-analysis

BackgroundGiant cell arteritis (GCA) is the most common primary systemic vasculitis in people 50 years of age and over, and it is considered a medical emergency due to the potential risk of permanent visual loss. Color Doppler ultrasound (CDU) of the temporal arteries is a rapid, noninvasive method to diagnose GCA. This study aims to determine the diagnostic accuracy of the halo sign in temporal arteries by CDU in people with suspected GCA.MethodsThe systematic literature review included the search for publications in the following electronic databases: PubMed, Embase, CENTRAL, LILACS, WHO ICTRP, ClinicalTrials.gov, gray literature up to December 2022, and no date or language restrictions were applied. We analyzed studies including patients over 50 years of age with suspected GCA evaluating CDU of temporal arteries as a diagnostic tool against clinical diagnosis as a standard reference. Paper titles and abstracts were selected by two investigators independently for all available records. The quality of the studies was assessed using the Quality of Diagnostic Accuracy Studies tool (QUADAS-2) and the R software (version 4.2.1) was used for data analysis. The protocol of this review is registered with PROSPERO (CRD42016033079).ResultsTwenty-two studies including 2893 participants with suspected GCA who underwent temporal artery CDU were evaluated. The primary analysis results showed a sensitivity of 0.76 [95% confidence interval (95 CI) 0.69–0.81] and specificity of 0.93 (95 CI 0.89–0.95) when the halo sign was compared to clinical diagnosis. The sensitivity value of 0.84 (95 CI 0.72–0.92) and specificity of 0.95 (95 CI 0.88–0.98) were found in five studies involving 1037 participants that analyzed the halo sign and temporal artery compression sign. A sensitivity of 0.86 (95 CI 0.78–0.91) and specificity of 0.95 (95 CI 0.89–0.98) were found in four studies with 603 participants where the halo sign was evaluated CDU on temporal and axillary arteries.ConclusionThe detection of the halo sign by CDU of temporal arteries has good accuracy for the diagnosis of cranial GCA. The compression sign in temporal arteries and the addition of axillary arteries assessment improves the diagnostic performance of CDU for GCA.Trial registrationPROSPERO CRD42016046860.

Quality standards for the care of people with giant cell arteritis in secondary care.

GCA is the commonest primary systemic vasculitis in adults, with significant health economic costs and societal burden. There is wide variation in access to secondary care GCA services, with 34% of hospitals in England not having any formal clinical pathway. Quality standards provide levers for change to improve services. The multidisciplinary steering committee were asked to anonymously put forward up to five aspects of service essential for best practice. Responses were qualitatively analysed to identify common themes, subsequently condensed into domain headings, and ranked in order of importance. Quality standards and metrics for each domain were drafted, requiring a minimum 75% agreement. 13 themes were identified from the initial suggestions. Nine quality standards with auditable metrics were developed from the top 10 themes. Patient Access, glucocorticoid use, pathways, ultrasonography, temporal artery biopsy, PET scan access, rheumatology/ophthalmology expertise, education, multidisciplinary working have all been covered in these quality standards. Access to care is a strand that has run through each of the developed standards. An audit tool was developed as part of this exercise. These are the first consensus auditable quality standards developed by clinicians from rheumatology and ophthalmology, nursing representatives and involvement of a patient charity. We hope that these standards will be adopted by commissioning bodies to provide levers for change from the improvement of patient care of individuals with GCA.