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Objectives The assessment of physical function is fundamental in the management of patients with idiopathic inflammatory myopathies (IIMs). We aimed to investigate the physical function of patients with IIMs compared with those with non-IIM autoimmune rheumatic diseases (AIRDs) utilizing Patient-Reported Outcome Measurement Information System (PROMIS) Physical Function (PF) data obtained in the COVAD study, an international self-reported e-survey assessing the safety of COVID-19 vaccines in AIRDs. Methods Demographics, AIRD diagnosis, disease activity, and PROMIS PF short form-10a data were extracted from the COVAD database. PROMIS PF-10a scores were compared between disease categories and stratified by disease activity. Factors affecting PROMIS PF-10a scores other than disease activity were identified by multivariable regression analysis in patients with inactive disease. Results A total of 1057 IIM patients, 3635 non-IIM AIRD patients and 3981 healthy controls (HCs) responded to the COVAD e-survey from April to August 2021. Using a binomial regression model, the predicted mean of PROMIS PF-10a scores was significantly lower in IIM patients compared with non-IIM AIRD patients or HCs [36.3 (95% CI 35.5, 37.1) vs 41.3 (95% CI 40.2, 42.5) vs 46.2 (95% CI 45.8, 46.6), P Conclusion Physical function is significantly impaired in IIMs compared with non-IIM AIRDs or HCs, even in patients with inactive disease. Our study highlights a critical need for better strategies to minimize functional disability in patients with IIMs.

COVID-19; Registries; Vaccination COVID-19; Registros; Vacunación COVID-19; Registres; Vacunació Vaccine hesitancy is considered a major barrier to achieving herd immunity against COVID-19. While multiple alternative and synergistic approaches including heterologous vaccination, booster doses, and antiviral drugs have been developed, equitable vaccine uptake remains the foremost strategy to manage pandemic. Although none of the currently approved vaccines are live-attenuated, several reports of disease flares, waning protection, and acute-onset syndromes have emerged as short-term adverse events after vaccination. Hence, scientific literature falls short when discussing potential long-term effects in vulnerable cohorts. The COVAD-2 survey follows on from the baseline COVAD-1 survey with the aim to collect patient-reported data on the long-term safety and tolerability of COVID-19 vaccines in immune modulation. The e-survey has been extensively pilot-tested and validated with translations into multiple languages. Anticipated results will help improve vaccination efforts and reduce the imminent risks of COVID-19 infection, especially in understudied vulnerable groups. HC is supported by the National Institution for Health Research Manchester Biomedical Research Centre Funding Scheme. The views expressed in this publication are those of the authors and not necessarily those of the NHS, the National Institute for Health Research or the Department of Health.

Objectives The COVID-19 vaccination in autoimmune diseases (COVAD) study aimed to assess short-term COVID-19 vaccination-related adverse events (AEs) in RA patients. Methods An online self-reported questionnaire (March–December 2021) was used to capture data related to COVID-19 vaccination-related AEs in RA, other autoimmune rheumatic diseases (AIRDs) (excluding RA and inflammatory myositis), non-rheumatic autoimmune diseases (nrAIDs) and healthy controls (HCs). Descriptive and multivariable regression analyses were performed. Results Of the 9462 complete respondents, 14.2% (n = 1347) had been diagnosed with RA; they had a mean (s.d.) age of 50.7 (13.7) years, 74.2% were women and 49.3% were Caucasian. In total, 76.9% and 4.2% of patients with RA reported minor and major AEs, respectively. Patients with active and inactive RA had similar AE and hospitalization frequencies. Overall, AEs were reported more frequently by BNT162b2 and mRNA-1273 recipients and less frequently by BBV152 recipients compared with the rest. Major AE and hospitalization frequencies were similar across recipients of different vaccines. Patients receiving methotrexate and hydroxychloroquine reported fewer minor AEs than those patients not on them. Compared with HCs and patients with other AIRDs, patients with RA reported similar total AEs, overall minor AEs, and hospitalizations. Compared with nrAIDs, patients with RA reported lower frequencies of overall AEs, minor AEs (both odds ratio [OR] = 0.7; 95% CI: 0.5, 0.9), and injection site pain (OR = 0.6; 95% CI: 0.5, 0.8) with similar major AE and hospitalization frequencies. Conclusion Despite the differences in AE frequency across different COVID-19 vaccines, all were well tolerated in patients with RA and were comparable to HCs, providing reassurance as to the safety of COVID-19 vaccination.

Objectives To define chronic ultrasound lesions of the axillary artery (AA) in long-standing giant cell arteritis (GCA) and to evaluate the reliability of the new ultrasound definition in a web-based exercise. Methods A structured Delphi, involving an expert panel of the Large Vessel Vasculitis subgroup of the Outcome Measures in Rheumatology (OMERACT) Ultrasound Working Group was carried out. The reliability of the new definition was tested in a 2-round web-based exercise involving 23 experts and using 50 still images each from AA of long-standing and acute GCA patients, as well as 50 images from healthy individuals. Results The final OMERACT ultrasound definition of chronic changes was based on measurement and appearance of the intima-media complex. The overall reliability of the new definition for chronic ultrasound changes in longstanding GCA of the AA was good to excellent with Light's kappa values of 0.79-0.80 for inter-reader reliability and mean Light's-kappa of 0.88 for intra-reader reliability. The mean inter-rater and intra-rater agreements were 86-87% and 92%, respectively. Good reliabilities were observed comparing the vessels with longstanding versus acute GCA with a mean agreement and kappa values of 81% and 0.63, respectively. Conclusion The new OMERACT ultrasound definition for chronic vasculitis of the AA in GCA revealed a good to excellent inter- and intra-reader reliability in a web-based exercise of experts.

The long-term mortality in giant cell arteritis (GCA) and polymyalgia rheumatica (PMR) is unexpectedly decreased or at least not increased regardless of several mortality risk factors that these diseases share with other chronic immune-mediated rheumatic diseases. The genetic and immunological profile of PMR/GCA patients is unique, therefore, the hypothesis that this profile provides some survival advantage to PMR/GCA patients should be considered. The longevity is a phenomenon that was demonstrated to be familial. The familial aggregation of longevity can be studied by analysis of life expectancy in family members. Here we test the hypothesis of the aggregation of an increased longevity in the families of PMR/GCA patients. We compared the age of death of 358 parents of 179 PMR and GCA patients with corresponding data retrieved from 506 parents of 253 randomly collected age and sex-matched controls. The number of nonagenarian (≥ 90-year -old) mothers of PMR/GCA patients was significantly higher (OR = 2.34, 95%CI 1.11–11.95, p p = 0.003). Our data suggest the familial aggregation of nonagenarians in PMR/GCA patients.

Objectives COVID-19 vaccines have been proven to be safe in the healthy population. However, gaps remain in the evidence of their safety in patients with systemic autoimmune and inflammatory disorders (SAIDs). COVID-19 vaccination-related adverse events (AEs) in patients with SAIDs and healthy controls (HC) seven days post-vaccination were assessed in the COVAD study, a patient self-reported cross-sectional survey. Methods The survey was circulated in early 2021 by >110 collaborators (94 countries) to collect SAID details, COVID-19 vaccination details and 7-day vaccine AEs, irrespective of respondent vaccination status. Analysis was performed based on data distribution and variable type. Results Ten thousand nine hundred respondents [median (interquartile range) age 42 (30–55) years, 74% females and 45% Caucasians] were analysed; 5867 patients (54%) with SAIDs were compared with 5033 HCs. Seventy-nine percent had minor and only 3% had major vaccine AEs requiring urgent medical attention (but not hospital admission) overall. Headache [SAIDs = 26%, HCs = 24%; odds ratio (OR) = 1.1 (95% CI: 1.03, 1.3); P = 0.014], abdominal pain [SAIDs = 2.6%, HCs = 1.4%; OR = 1.5 (95% CI: 1.1, 2.3); P = 0.011], and dizziness [SAIDs = 6%, HCs = 4%; OR = 1.3 (95% CI: 1.07, 1.6); P = 0.011], were slightly more frequent in SAIDs. Overall, major AEs [SAIDs = 4%, HCs = 2%; OR = 1.9 (95% CI: 1.6, 2.2); P Conclusion Vaccination against COVID-19 is safe in SAID patients. SAIDs were at a higher risk of major AEs than HCs, though absolute risk was small. There are small differences in minor AEs between vaccine types in SAID patients.

ObjectivesTo develop an Outcome Measures in Rheumatology (OMERACT) ultrasonography score for monitoring disease activity in giant cell arteritis (GCA) and evaluate its metric properties.MethodsThe OMERACT Instrument Selection Algorithm was followed. Forty-nine members of the OMERACT ultrasonography large vessel vasculitis working group were invited to seven Delphi rounds. An online reliability exercise was conducted using images of bilateral common temporal arteries, parietal and frontal branches as well as axillary arteries from 16 patients with GCA and 7 controls. Sensitivity to change and convergent construct validity were tested using data from a prospective cohort of patients with new GCA in which ultrasound-based intima–media thickness (IMT) measurements were conducted at weeks 1, 3, 6, 12 and 24.ResultsAgreement was obtained (92.7%) for the OMERACT GCA Ultrasonography Score (OGUS), calculated as follows: sum of IMT measured in every segment divided by the rounded cut-off values of IMTs in each segment. The resulting value is then divided by the number of segments available. Thirty-five members conducted the reliability exercise, the interrater intraclass correlation coefficient (ICC) for the OGUS was 0.72–0.84 and the median intrareader ICC was 0.91. The prospective cohort consisted of 52 patients. Sensitivity to change between baseline and each follow-up visit up to week 24 yielded standardised mean differences from −1.19 to −2.16, corresponding to large and very large magnitudes of change, respectively. OGUS correlated moderately with erythrocyte sedimentation rate, C reactive protein and Birmingham Vasculitis Activity Score (corrcoeff0.37–0.48).ConclusionWe developed a provisional OGUS for potential use in clinical trials.

ObjectivesThis study aimed to determine the prevalence of ultrasound-detected tendon abnormalities in healthy subjects (HS) across the age range.MethodsAdult HS (age 18–80 years) were recruited in 23 international Outcome Measures in Rheumatology ultrasound centres and were clinically assessed to exclude inflammatory diseases or overt osteoarthritis before undergoing a bilateral ultrasound examination of digit flexors (DFs) 1–5 and extensor carpi ulnaris (ECU) tendons to detect the presence of tenosynovial hypertrophy (TSH), tenosynovial power Doppler (TPD) and tenosynovial effusion (TEF), usually considered ultrasound signs of inflammatory diseases. A comparison cohort of patients with rheumatoid arthritis (RA) was taken from the Birmingham Early Arthritis early arthritis inception cohort.Results939 HS and 144 patients with RA were included. The majority of HS (85%) had grade 0 for TSH, TPD and TEF in all DF and ECU tendons examined. There was a statistically significant difference in the proportion of TSH and TPD involvement between HS and subjects with RA (HS vs RA pConclusionsUltrasound-detected TSH and TPD abnormalities are rare in HS and can be regarded as markers of active inflammatory disease, especially in newly presenting RA.

IntroductionFast-track clinics (FTC) have been introduced in different fields and have been reporting significant outcomes in terms of reducing mortality, morbidity, and financial costs. To date, scarce evidence is available for FTC specific for patients suspected of polymyalgia rheumatica (PMR). The primary aim of our paper is to provide an overview of the clinical impact of PMR on patients and the healthcare system by analysing multiple aspects: the median time from onset of symptoms to diagnosis and the burden of the disease both on the healthcare system costs and on patients’ quality of life (QoL). Secondarily, based on these data, we aim to discuss the potential advantages and feasibility of a PMR FTC in everyday clinical practice.Material and methodsWe performed a narrative non-systematic review (PRISMA protocol not followed) of PubMed and Medline (OVID interface) with the following MeSH terms: [polymyalgia rheumatica AND diagnosis OR diagnosis, delayed OR patient care OR early diagnosis OR length of stay OR costs OR healthcare system OR quality of life] or [polymyalgia rheumatica AND glucocorticoids AND side effects]. We decided to exclude every paper that did not report raw data in terms of diagnostic time or delay, hospitalization rate, socio-economic costs on the healthcare system, patients’ QoL, and glucocorticoids-related events in PMR patients. Papers focused primarily on giant cell arteritis patients with overlapping PMR were also excluded. Abstract archives of the European League Against Rheumatism (EULAR) and the American College of Rheumatology (ACR) congresses of the last 10 years were screened and included in the search if raw data were available. Each paper’s reference list was scanned for additional publications meeting this study’s aims. When papers reported data partially presented in previous articles, we opted to use the most recently published data.ResultsAccording to our literature review, a PMR FTC might lighten the burden of the disease. Nevertheless, its feasibility depends mostly on the resources of the national health system and of the territorial health district, which are heterogeneously limited. The usefulness of PMR FTCs depends on closer collaboration with the general practitioner because he/she is the first clinician to visit patients with PMR.ConclusionsPolymyalgia rheumatica fast-track clinics might lighten the burden of the disease. However, it has some limits that should carefully assessed in planning health policies.

Background and Objectives: Laboratory liver abnormalities can be observed in patients affected with polymyalgia rheumatica (PMR) and/or giant cell arteritis (GCA), especially with a cholestatic pattern. The first objective of our review article is to discuss the potential link between antimitochondrial antibodies (AMA) and/or primary biliary cholangitis (PBC) and PMR/GCA, according to the evidences of literature. The second objective is to discuss the association of PMR/GCA with the other rheumatic diseases having PBC as a common manifestation. Materials and Methods: A literature search was performed on PubMed and Medline (OVID interface) using these terms: polymyalgia rheumatica, giant cell arteritis, antimitochondrial antibodies, primary biliary cholangitis, primary Sjogren’s syndrome, systemic sclerosis, and systemic lupus erythematosus. The search was restricted to all studies and case reports published in any language. Reviews, conference abstracts, comments, and non-original articles were excluded; however, each review’s reference list was scanned for additional publications meeting this study’s aim. When papers reported data partially presented in previous articles, we referred to the most recent published data. Results and Conclusions: Our literature search highlighted that cases reporting an association between AMA, PBC and PMR/GCA were very uncommon; AMA antigenic specificity had never been detected and biopsy-proven PBC was reported only in one patient with PMR/GCA. Finally, the association of PMR/GCA with autoimmune rheumatic diseases in which PBC is relatively common was anecdotal.

The coronavirus disease-2019 (COVID-19) pandemic continues to be a cause of unprecedented global morbidity and mortality. Whilst COVID-19 vaccination has emerged as the only tangible solution to reducing poor clinical outcomes, vaccine hesitancy continues to be an obstacle to achieving high levels of vaccine uptake. This represents particular risk to patients with autoimmune diseases, a group already at increased risk of hospitalization and poor clinical outcomes related to COVID-19 infection. Whilst there is a paucity of long-term safety and efficacy data of COVID-19 vaccination in patients with autoimmune diseases, the current evidence strongly suggests that the benefits of vaccination outweigh the risks of adverse effects and disease flares. Herein, we report the protocol of the COVID-19 Vaccination in Autoimmune Diseases (COVAD) study, an ongoing international collaborative study involving 29 countries and over 110 investigators. Supplementary Information The online version contains supplementary material available at 10.1007/s00296-021-05046-4.

IntroductionIn 1979, Bird et al. proposed depression as a diagnostic criterion for polymyalgia rheumatica (PMR). More recently, the significance of depression in PMR patients has been re-proposed, , and some researchers have suggested that PMR may increase the risk of depression. The aim of our article is to evaluate the relationship between PMR and depression.Material and methodsSystematic literature searches were performed on 19th and 20th May 2020 based on Preferred Reporting Items for Systematic reviews and Meta-Analyses (PRISMA) guidelines. The search was restricted to all studies and case reports with English abstract, published in any language, since 1979 (when depression was first proposed as a diagnostic criterion for PMR) describing the association of PMR with depression. Exclusion criteria were as follows: reviews, conference abstracts, comments, non-original articles; and articles discussing giant cell arteritis (GCA) and PMR when data and observations for the two conditions were not clearly subdivided.ResultsThe initial search yielded 812 papers, of which 115 duplicates were removed. A total of 697 articles had a first screening and 506 were excluded based on title and abstract reviews; 117 articles underwent full-length scrutiny, and 99 full-text articles were excluded because they did not meet the inclusion and exclusion criteria (reviews and comments = 58; articles with outcome of interest not reported = 34; low-quality articles = 7). At least, 18 articles were included in this review.ConclusionsThe review did not find any studies that clarified the prevalence rates of depression in patients with PMR. Furthermore, the studies reviewed did not offer any clarity as to whether patients suffered from just depressive symptoms or clinical depression, and that accepted diagnostic criteria for depression had not been employed, indicating that a robust method for diagnosing depression had not been employed. Collaboration of different professionals should be improved through shared guidelines.

Objective.To test the reliability of Outcome Measures in Rheumatology Clinical Trials (OMERACT) consensus-based ultrasound definitions for normal and vasculitic temporal and axillary arteries in patients with giant cell arteritis (GCA) and in controls.Methods.A preliminary 1-day meeting and a full 3-day meeting fulfilling OMERACT Ultrasound Group guidelines were held. Temporal and axillary arteries were examined at 2 timepoints by 12 sonographers on 4 patients with GCA and 2 controls. The aim was to test inter- and intrareader reliability for normal findings, halo sign, and compression sign. In both meetings, patients had established GCA. Pathology was more recent in the full meeting, which was preceded by 6 h of training. Scanning time was 15–20 min instead of 10–13 min.Results.In the preliminary exercise, interreader reliabilities were fair to moderate for the overall diagnosis of GCA (Light κ 0.29–0.51), and poor to fair for identifying vasculitis in the respective anatomical segments (Light κ 0.02–0.46). Intrareader reliabilities were moderate (Cohen κ 0.32–0.64). In the main exercise, interreader reliability was good to excellent (Light κ 0.76–0.86) for the overall diagnosis of GCA, and moderate to good (Light κ 0.46–0.71) for identifying vasculitis in the respective anatomical segments. Intrareader reliability was excellent for diagnosis of GCA (Cohen κ 0.91) and good (Cohen κ 0.71–0.80) for the anatomical segments.Conclusion.OMERACT-derived definitions of halo and compression signs of temporal and axillary arteries are reliable in recent-onset GCA if experienced sonographers (> 300 examinations) have 15–20 min for a standardized examination with prior training and apply > 15 MHz probes.

Silent vasculitis may accompany polymyalgia rheumatica (PMR), resulting in complications such as vision deterioration. Reduced visual field is a possible manifestation. A 66-year-old man was diagnosed with PMR and treated accordingly (prednisone maximum 15 mg/day) at a tertiary center, with resolution of his symptoms. After 18 months, he suddenly experienced right eye vision loss. Visual field of his left eye was peripherally limited (Figure 1). Color Doppler ultrasound …

Background: Tenosynovitis (TS) is a common, often clinically undetectable finding in Rheumatoid Arthritis (RA). Recent data showed TS on ultrasound (US) has a role in predicting outcome in early disease and flare in clinical remission. However data is limited on US measured TS in healthy subjects (HS), none specifically encompassing the older age range when RA commonly presents. Objectives: This OMERACT study aimed to determine prevalence of US measured tendon abnormalities in HS throughout the age range. Methods: Adult HS without: joint pain (VAS A comparison cohort of DMARD-naive patients with RA (ACR-EULAR 2010 and/or 1987 criteria) at presentation was taken from the Birmingham Early Arthritis (BEACON) inception cohort, who underwent identical tendon US assessment. They were grouped into ≤12 and > 12 weeks from symptom onset. Results: Data from 899 HS and 144 RA patients were included. Prevalence of TSH and particularly PD abnormalities in HS was very low at all ages, and was all grade 1 except in one individual ECU tendon. ECU TSH grade≥1 was more common than DF grade≥1 in the older HS groups, and less common in the 18-39 age group (p=0.011). TSH and PD of grade ≥1 were common in RA patients, with DF PD abnormalities more common in early disease (p=0.02). Conclusion: Low prevalence of TSH or PD abnormalities in tendons of HS even in old age suggests US determined TS will be a robust tool in clinically managing RA. References: [1] Naredo E, D’Agostino MA, et al. Reliability consensus-based US score TS RA. ARD.2013;72(8):1328-34 Disclosure of Interests: Jeanette Trickey: None declared, Ilfita Sahbudin: None declared, Alessandra Bortoluzzi: None declared, Annamaria Iagnocco: None declared, Carlos Pineda: None declared, Cesar Sifuentes-Cantu: None declared, Coziana Ciurtin: None declared, Cristina Reategui Sokolova: None declared, Daniela Fodor: None declared, Ellen-Margrethe Hauge: None declared, Esperanza Naredo Consultant for: Abbvie, Speakers bureau: AbbVie, Roche, Bristol-Myers Squibb, Pfizer, UCB, Lilly, Novartis, Janssen, and Celgene GmbH, Florentin Ananu Vreju Consultant for: abbvie and novartis, Speakers bureau: abbvie, novartis, pfizer, sandoz, eli lilly, ucb pharma, Garifallia Sakellariou: None declared, George Bruyn : None declared, Georgios Filippou Speakers bureau: Laborest, Abbvie, BMS, Sanofi, Giuliana M.C. La Paglia : None declared, Gustavo Leon: None declared, Helen Keen: None declared, Hilde Berner Hammer Grant/research support from: AbbVie, Pfizer and Roche, Paid instructor for: AbbVie, Pfizer, UCB, Novartis, Roche, Speakers bureau: AbbVie, Pfizer, UCB, Novartis, Roche, Ilaria Tinazzi: None declared, Irene Azzolin: None declared, Jacek Flicinski: None declared, James Wilton: None declared, Kei Ikeda: None declared, Lene Terslev Speakers bureau: Speakers fee from : Roche, Novartis, Pfizer, MSD, BMS, Celgene, Mads Ammitzboll-Danielsen: None declared, Mads Nyhuus Bendix Rasch: None declared, Marcin Milchert: None declared, Maria Stoenoiu Grant/research support from: Abbvie, Roche, Wyeth, Marion Kortekaas: None declared, Marwin Gutierrez: None declared, Mihaela Maruseac: None declared, Mohammed A Mortada: None declared, Philippe Carron: None declared, Rositsa Karalilova: None declared, Ruth Wittoek: None declared, Sarah Ohrndorf: None declared, Takeshi Suzuki: None declared, Teodora Serban: None declared, Maria-Antonietta d’Agostino: None declared, Andrew Filer: None declared

Background Autoinflammatory diseases (AIDs) cover a spectrum of diseases, which lead to chronic or recurrent inflammation caused by activation of the innate immune system, typically in the absence of high titre autoantibodies.1 The most common monogenic AIDs are cryopyrin-associated periodic syndromes (CAPS), familial Mediterranean fever (FMF) and tumour necrosis factor receptor-associated periodic fever syndrome (TRAPS). In hereditary AIDs, chronic and recurrent inflammation can lead to both acute disease and irreversible damage.2 Objectives To describe irreversible damage and diagnostic delay of AID in Polish adult patients (age≥18). Methods We rewieved the records of 11 Caucasian (2 women, 9 men) and one female patient with Middle Eastern ancestry followed in our center from Aug 2012 to Jan 2019. We analysed demographic features, diagnostic delay, previous diagnosis and organ damage3. Results Eleven patients had documented variant mutation and AID diagnosis (6-TRAPS, 4-CAPS, 1-FMF). One patient with AA amyloidosis had negative genetic results. The mean age of the patients at the time of disease onset was 9,1 years (0,1- 37) and 33 years (16-62) at diagnosis, In all Caucasian patients diagnosis was established after the age of 18. Previous diagnosis was: JIA (2), AOSD (2), UCTD (4), vasculitis (1), seronegative RA (1), FMF (3 finaly confirmed TRAPS) and allergy (3). Prior treatment included GCS (12/12), cDMARDS (7), antimalarials (6), colchcine (4), bDMARDS: tocilizumab-1, etanercept-2, adalimumab-1, CYC (2).After diagnosis 11 patients received IL-1 inhibitor, ankinra, Only one, 62 year old woman with TRAPS, did not need biological treatment. Only 3 TRAPS patients were free from damage accrual. We documented following damage categories: renal/amyloidosis (5 male cases), developmental (3 growth retardation), ear (severe hearing loss -3 CAPS cases). CINCA male patient at age 29 had multiple organ damage: neurological (epilepsy), severe hearing loss, severe ocular involvement (including optic nerve athrophy), joint restriction and deformity leading to complete physical disability. One FMF woman was adviced elective abortion, one MWS had miscarriage. Two of 3 women had history of malignancy (one: breast cancer and ovarian cancer, and another thyroid cancer). No any mental retardation occured. During follow up 2 male patients with extensive amyloidosis died despite anakinra treatment. Conclusion AID are misdiagnosed and have high risk of damage. We advocate the need for efforts in order to increase awarness of AID to reduce the diagnostic delay. The frequency of malignancy in AID should be addressed in future international collaborative studies. References [1] Masters SL, et al. Horror autoinflammaticus: the molecular pathophysiology of autoinflammatory disease. Annu Rev Immunol 2009;27:621–68. [2] Federici S, et al. Evidence-based provisional clinical classification criteria for autoinflammatory periodic fevers. Ann Rheumatic Dis 2015;74:799–805. [3] ter Haar NM, et al. Development of the autoinfammatory disease damage index. Ann Rheum Dis 2017;76:821–830 Disclosure of Interests None declared

Polymyalgia rheumatica (PMR) is a unique disease of elderly people, traditionally diagnosed based on a clinical picture. A typical case is a combination of severe musculoskeletal symptoms and systemic inflammatory response with spectacular response to corticosteroids treatment. The severity of symptoms may be surprising in older patients where immunosenescence is normally expected. However, PMR may be diagnosed in haste if there is a temptation to use this diagnosis as a shortcut to achieve rapid therapeutic success. Overdiagnosis of PMR may cause more problems compared to underdiagnosis. The 2012 PMR criteria proposed by European League against Rheumatism/American College of Rheumatology aim to minimize the role of clinical intuition and build on more objective features. However, questions arise if this is possible in PMR. This has been discussed in this review.

Objective Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV) are rare small to medium-size vessel systemic diseases. As their clinical picture, organ involvement, and factors influencing outcome may differ between countries and geographical areas, we decided to describe a large cohort of Polish AAV patients coming from several referral centers-members of the Scientific Consortium of the Polish Vasculitis Registry (POLVAS). Methods We conducted a systematic multicenter retrospective study of adult patients diagnosed with AAV between Jan 1990 and Dec 2016 to analyze their clinical picture, organ involvement, and factors influencing outcome. Patients were enrolled to the study by nine centers (14 clinical wards) from seven Voivodeships populated by 22.3 mln inhabitants (58.2% of the Polish population). Results Participating centers included 625 AAV patients into the registry. Their distribution was as follows: 417 patients (66.7%) with GPA, 106 (17.0%) with MPA, and 102 (16.3%) with EGPA. Male-to-female ratios were almost 1:1 for GPA (210/207) and MPA (54/52), but EGPA was twice more frequent among women (34/68). Clinical manifestations and organ involvement were analyzed by clinical phenotype. Their clinical manifestations seem very similar to other European countries, but interestingly, men with GPA appeared to follow a more severe course than the women. Fifty five patients died. In GPA, two variables were significantly associated with death: permanent renal replacement therapy (PRRT) and respiratory involvement (univariate analysis). In multivariate analysis, PRRT (OR = 5.3; 95% confidence interval (CI) = 2.3–12.2), respiratory involvement (OR = 3.2; 95% CI = 1.06–9.7), and, in addition, age > 65 (OR = 2.6; 95% CI = 1.05–6.6) were independently associated with death. In MPA, also three variables were observed to be independent predictors of death: PRRT (OR = 5.7; 95% CI = 1.3–25.5), skin involvement (OR = 4.4; 95% CI = 1.02– 19.6), and age > 65 (OR = 6.3; 95% CI = 1.18–33.7). Conclusions In this first multicenter retrospective study of the Polish AAV patients, we have shown that their demographic characteristics, disease manifestations, and predictors of fatal outcome follow the same pattern as those from other European countries, with men possibly suffering from more severe course of the disease.

Objectives: To define the elementary ultrasound (US) lesions in giant cell arteritis (GCA) and to evaluate the reliability of the assessment of US lesions according to these definitions in a web-based reliability exercise.Methods: Potential definitions of normal and abnormal US findings of temporal and extracranial large arteries were retrieved by a systematic literature review. As a subsequent step, a structured Delphi exercise was conducted involving an expert panel of the Outcome Measures in Rheumatology (OMERACT) US Large Vessel Vasculitis Group to agree definitions of normal US appearance and key elementary US lesions of vasculitis of temporal and extracranial large arteries. The reliability of these definitions on normal and abnormal blood vessels was tested on 150 still images and videos in a web-based reliability exercise.Results: Twenty-four experts participated in both Delphi rounds. From originally 25 statements, nine definitions were obtained for normal appearance, vasculitis and arteriosclerosis of cranial and extracranial vessels. The 'halo' and 'compression' signs were the key US lesions in GCA. The reliability of the definitions for normal temporal and axillary arteries, the 'halo' sign and the 'compression' sign was excellent with inter-rater agreements of 91-99% and mean kappa values of 0.83-0.98 for both inter-rater and intra-rater reliabilities of all 25 experts.Conclusions: The 'halo' and the 'compression' signs are regarded as the most important US abnormalities for GCA. The inter-rater and intra-rater agreement of the new OMERACT definitions for US lesions in GCA was excellent.

Background: Th e incidence rate of giant cell arteritis (GCA) in Department of Rheumatology, Internal Medicine and Geriatrics was raised 10 folds between 2000 and 2016. Objectives: In this presentation a practical question: ”how to increase GCA diagnostic rate?” was addressed, and illustrated with some clinical cases. Methods: GCA referrals to Department of Rheumatology in Szczecin were analyzed. Additionally GCA epidemiology in Poland was analyzed based on national insurance registry and compared with distribution of fast track GCA clinics. Results: Increase of diagnostic rate was due to mainly 2 factors: 1. Development of eff ective temporal / large vessels visualization techniques and introduction of fast track GCA diagnostics. 2. Better cooperation with ophthalmologist and other specialists (Table 1.) Visualization techniques introduced were temporal/large arteries Doppler ultrasound (fast track) sometimes supported by large arteries computed tomography, occasionally by temporal artery biopsy and marginally by PET-CT. Based on national insurance registry, that we’ve analyzed, GCA diagnostic rate in Poland improved in recent years. Th is seems to correlate with increased interest in GCA in some sites that organized fast track GCA clinics. Conclusions: Increase of GCA diagnostic rate requires further efforts. References: 1. Atlas of ultrasound application in large vessel arteritis: giant cell arteritis and Takayasu arteritis., Milchert M, Diamantopoulos A, Brzosko M; Wydawnictwo Pomorskiej Akademii Medycznej, Szczecin, 2015

Background Color duplex ultrasonography (CDU) is most promising tool for the assessment of large vessel giant cell arteritis (LV-GCA). There is a need to define ultrasound findings consistent with the diagnosis of GCA. Objectives We aimed to score intima-media thickness (IMT) reference ranges for LV-GCA in polish patients. Methods 214 patients suspected for GCA and evaluated with CDU were included in the study and followed up for min 9 months. Large vessel CDU, together with arteritis/non-arteritis categorization, were performed before or within 1 week after treatment initiation by a single physician. Vasculitis was defined as hypoechoic, homogenous, increase of IMT with distorted wall structure resulting in no clear intima-media structure, over long distance (not limited only to the place of arterial bifurcations). ROC curves were calculated. Results GCA was diagnosed in 81 patients, polymyalgia rheumatica (PMR) in 131 (characteristics – Table 1). Extracranial LV-GCA was diagnosed in 43 patients: axillary vasculitis in 23 patients, common carotid artery (CCA) – 24, subclavian – 18, superficial femoral – 11, brachial (all spreading per continuum from axillary arteritis) – 8. In 83 remaining patients other diagnosis was confirmed, and they served as non-GCA/PMR controls. Mean IMT in LV-GCA was significantly higher versus controls and isolated PMR (Fig. 1). IMT in GCA was not significantly influenced by gender, hypertension and smoking in contrast with IMT in controls. Proposed cut off values for IMT depicting vasculitis in GCA patients are presented in Table 2. 100% specificity for vasculitis (vs GCA without large vessel vasculitis) was reached with axillary IMT of 1.06 mm (62% sens.), subclavian – 1.35 mm (38% sens.), superficial femoral – 1.55 mm (60% sens.), CCA – 1.27 mm (22% sens.). Conclusions We demonstrated that cut off values may discriminate between GCA and its mimics as well as between presence and lack of vasculitis in different arteries in GCA. References Milchert M, Diamantopoulos A, Brzosko M. Atlas of ultrasound application in large vessel arteritis: giant cell arteritis and Takayasu arteritis. Wydawnictwo Pomorskiej Akademii Medycznej, Szczecin, 2016. Disclosure of Interest None declared

Biosimilars are follow-up versions of innovative medicines with proved high similarity to the reference product. There may be some minor differences in clinically inactive components of a molecule which can be clinically significant, but they do not substantially affect the high resemblance to the original drug in their safety and potency. Introduction of the biosimilars brings hope to reduce the cost of treatment, so their availability will increase further as the expiration of patents on biotechnologically produced drugs will follow, which is currently taking place. There are new biosimilar drugs being introduced recently; these are monoclonal antibodies. They are much more structurally complicated compared to the first biotech drugs such as insulin, and their production processes are much more complex. Biosimilars as the end products of such sophisticated technology are affected by many variables. It is not possible to create "biogenerics," but only biosimilar drugs. Therefore, despite their similarities to the reference molecules, biosimilars are too different to forego clinical trials. Clinical trials of biosimilars need to be much broader than for generics, but they are limited compared to the reference biologicals' testing. Placebo controlled clinical trials are not required before registering a new biosimilar. Immunogenicity is a major source of concern related to biosimilars, although there may be the same problem with the original drugs.

We have read with great interest the observation of Dasgupta et al. 1 We would like to propose an extended definition of slope sign in giant cell arteritis (GCA) formerly named slide sign,2,3 and present our method of assessing it by using the anteromedial ultrasound examination method of the large supraaortic vessels.4 Slope sign is a pathologically increased intima-media thickness (IMT)5 that spreads over a long arterial segment and slides down to a normal brachial artery where a normal intima-media structure (double line) is observed. This definition contains a description of pathological findings in the axillary artery—a typical location for vasculitis in GCA, contrasting with normal findings in brachial artery—which is usually not involved by vasculitis.6 Slope sign may not be limited to the place of arterial bifurcation of the subscapular artery, which is a typical location of atherosclerotic plaque. A transition zone …