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Objectives To explore current management practices for polymyalgia rheumatica (PMR) by general practitioners (GPs) and rheumatologists including implications for clinical trial recruitment. Methods An English language questionnaire was constructed by a working group of rheumatologists and GPs from 6 countries. The questionnaire focused on: 1: Respondent characteristics, 2: Referral practices, 3: Treatment with glucocorticoids, 4: Diagnostics, 5: Comorbidities, and 6: Barriers to research. The questionnaire was distributed to rheumatologists and GPs worldwide via members of the International PMR/giant cell arteritis Study Group. Results In total, 394 GPs and 937 rheumatologists responded to the survey. GPs referred a median of 25% of their suspected PMR patients for diagnosis and 50% of these were returned to their GP for management. In general, 39% of rheumatologists evaluated patients with suspected PMR >2 weeks after referral, and a median of 50% of patients had started prednisolone before rheumatologist evaluation. Direct comparison of initial treatment showed that the percentage prescribing >25 mg prednisolone daily for patients was 30% for GPs and 12% for rheumatologists. Diagnostic imaging was rarely used. More than half (56%) of rheumatologists experienced difficulties recruiting people with PMR to clinical trials. Conclusion This large international survey indicates that a large proportion of people with PMR are not referred for diagnosis, and that the proportion of treatment naïve patients declined with increasing time from referral to assessment. Strategies are needed to change referral and management of people with PMR, to improve clinical practice and facilitate recruitment to clinical trials.

ObjectivesTo characterise factors associated with permanent vision loss (PVL) and potential reasons for the therapeutic delay contributing to PVL in giant cell arteritis (GCA).MethodsRetrospective analysis of GCA patients diagnosed at the University Hospital Basel between December 2006 and May 2021.ResultsOf 282 patients with GCA (64% females), 49 (17.4%) experienced PVL. In 43/49 (87.8%) PVL occurred before treatment. Of these, 24 (55.8%) patients had first non-ocular symptoms and eventually sought consultation when PVL occurred in a median of 21 (IQR 14.75–31.0) days after the first symptoms. Only five of the 24 patients had consulted a physician before PVL, but GCA diagnosis was missed. Treatment was initiated rapidly after diagnosis (median 1 day (IQR 0.0–7.0)). PVL on therapy occurred in six patients in a median of 40 (IQR 20.5–67.3) days after treatment started. In two of those, glucocorticoids were tapered too quickly.In multivariable analysis, patients with PVL were older (OR 1.17, 95% CI 1.07 to 1.29, p=0.001) and reported more frequently jaw claudication (OR 3.52, 95% CI 1.02 to 13.16, p=0.051). PVL was present in 18 (42.9%) of the 42 patients with vasculitic ultrasound findings in all six temporal artery segments. The incidence of PVL over 15 years did not decline (Spearman rank=0.3, p=0.68).ConclusionThe prevalence of GCA-associated PVL remains high. Associated factors were advanced age, jaw claudication and ultrasound findings consistent with vasculitis in all six temporal artery segments. Despite preceding non-ocular GCA symptoms weeks before the onset of PVL, most patients were not seen by a rheumatologist before PVL occurred.

Background:GCA is characterized by cranial symptoms but imaging techniques show that patients with non-specific symptoms such as systemic inflammation or PMR may have undiagnosed large vessel (LV) GCA1. Although silent GCA in patients with clinically isolated PMR may have consequences for patients’ outcome, little is known about its prevalence and characteristics of affected patients.Objectives:To review data on the prevalence of silent GCA in newly diagnosed PMR patients without cranial GCA symptoms and to analyze which characteristics are associated with vascular involvement among PMR patients.Methods:We systematically screened PubMed, Embase and Web of Science databases and included studies screening for GCA in steroid naïve PMR patients without cranial symptoms consistent with GCA. Authors of the publications that used PET for vasculitis screening were invited to share their individual patient data (IPD) for a meta-analysis. We sought to define patient characteristics that were associated with vasculitis using univariable mixed effects logistic regression models with vascular involvement as the outcome, missing values were imputed using multilevel joint modeling multiple imputation. To fit a multivariable model with the candidate predictors we excluded variables that were hypothesized to have less medical relevance for the outcome and highly correlated inflammation markers (ESR, Lc).Results:Out of the 3047 studies screened independently by 2 authors (DG and TD), 13 fulfilled the inclusion criteria. These studies (published 1963-2019) reported on 543 PMR patients examined by temporal artery biopsy (n=175), ultrasound (n=110), PET or PET-CT (n=258). 115 PMR patients were diagnosed with GCA (21.2%), with prevalence ranging from 0-92%.We collected IPD for 243 patients from 4 cohorts using PET and 3 using PET/CT for GCA diagnosis. The overall median age of patients was 72.3 years (IQR 66.4-78.0) and vasculitis was found in 65 patients (26.7%) (table 1).Table 1.OverallPMRPMR+GCAn (%)243178 (73.3)65 (26.7)Female sex (%)146 (60.1)98 (55.1)48 (73.8)Shoulder girdle pain (%)236 (97.1)174 (97.8)62 (95.4)Pelvic girdle pain (%)174 (71.6)127 (71.3)47 (72.3)Inflammatory back pain (%)No107 (44.0)83 (46.6)24 (36.9)Yes106 (43.6)70 (39.3)36 (55.4)Lower limb pain (%)No87 (35.8)61 (34.3)26 (40.0)Yes81 (33.3)68 (38.2)13 (20.0)Weight loss (%)112 (46.1)78 (43.8)34 (52.3)CRP (mg/l) (median [IQR])46.0 [19.0, 77.7]44.0 [16.9, 74.2]52.0 [27.9, 85.0]ESR (mm/h) (mean (SD))65.2 (30.3)62.7 (30.2)72.3 (29.7)Hemoglobin (g/dl) (mean (SD))12.1 (1.5)12.2 (1.5)11.7 (1.6)Thrombocytes (1e+09/ml) (mean (SD))341.9 (106.3)323.9 (103.2)375.8 (104.6)In the univariable analyses the following factors were most strongly associated with vasculitic PET findings: female sex (OR 2.31, CI 1.17-4.58), inflammatory back pain (OR 2.73, CI 1.32-5.64), temperature >37° (OR 1.83, CI 0.90-3.7), weight loss (OR 1.83, CI 0.96-3.51), thrombocytosis (i.e., patients with a thrombocyte count 1 SD above mean have an OR of 1.51, CI 1.05-2.18), anemia (i.e., 1 g/dl decrease in Hb below mean corresponds to an OR of 1.25, CI 1.00-1.56). Patients with lower limb pain were less likely to have vasculitis (OR 0.43, CI 0.19–0.95). The estimated ORs were very similar in the multivariable model although the 95%CIs became wider.Conclusion:Although the prevalence across published studies showed substantial variation, 6 out of 13 studies reported a prevalence of silent GCA in 18-40% of all PMR patients. The exploratory analysis of the collected IPD identified female sex, inflammatory back pain, fever, weight loss, absence of lower leg pain, thrombocytosis and anemia as factors associated with LV-GCA. These findings should be validated in future prospective cohort studies. The presence or absence of these factors may further aid in diagnosing LV-GCA in PMR patients.References:[1]Buttgereit F, Dejaco C, Matteson EL, Dasgupta B. Polymyalgia Rheumatica and Giant Cell Arteritis: A Systematic Review. JAMA. 2016 Jun 14;315(22):2442–58.Acknowledgements:The study is funded by the “Schweizerische Stiftung für die Erforschung der Muskelkrankheiten (SSEM)”.Disclosure of Interests:Daniele Silvio Gozzoli: None declared, Andrea Hemmig: None declared, Lars Hemkens: None declared, Laura Werlen: None declared, Hannah Ewald: None declared, Christoph Berger: None declared, Diego Kyburz Grant/research support from: DK reports personal fees from Abbvie, Gilead, Lilly, Novartis and Pfizer, outside of the submitted work, Stephan Imfeld: None declared, Markus Aschwanden: None declared, Mihaela Stegert: None declared, Dario Camellino: None declared, Marco Amedeo Cimmino: None declared, Corrado Campochiaro Grant/research support from: personal fees from Roche, Alessandro Tomelleri: None declared, Liesbet Henckaerts: None declared, Daniel Blockmans Speakers bureau: Paid speaker for Roche, Consultant of: Paid consultant for Roche, Patricia Moya: None declared, Hector Corominas: None declared, Russell Buchanan: None declared, Claire Owen Speakers bureau: CO has received speaking honoraria from Roche, Janssen, Novartis and Pfizer, and meeting sponsorship from Roche, UCB and Janssen, Yannick van Sleen: None declared, Elisabeth Brouwer Speakers bureau: E. Brouwer as an employee of the UMCG received speaker fees and consulting fees from Roche in 2017, 2018 which were paid to the UMCG, Consultant of: E. Brouwer as an employee of the UMCG received speaker fees and consulting fees from Roche in 2017, 2018 which were paid to the UMCG, Hiroyuki Ymashita: None declared, Thomas Daikeler: None declared

ObjectivesWe evaluated the feasibility of a rapid glucocorticoid tapering regimen to reduce glucocorticoid exposure in patients with giant cell arteritis (GCA) treated with glucocorticoids only.MethodsNewly diagnosed patients with GCA treated with a planned 26-week glucocorticoid tapering regimen at the University Hospital Basel were included. Data on relapses, cumulative steroid doses (CSD) and therapy-related adverse effects were collected from patients’ records.ResultsOf 47 patients (64% women, median age 72 years), 32 patients (68%) had relapsed. Most relapses were minor (28/32) and 2/3 of those were isolated increased inflammatory markers (19/32). Among major relapses, one resulted in permanent vision loss. The median time until relapse was 99 days (IQR 71–127) and median glucocorticoid dose at relapse was 8 mg (IQR 5–16). Nine of 47 patients stopped glucocorticoids after a median duration of 35 weeks and did not relapse within 1 year. Median CSD at 12 months was 4164 mg which is lower compared with published data. Glucocorticoid-associated adverse effects occurred in 40% of patients, most frequently were new onset or worsening hypertension (19%), diabetes (11%) and severe infections (11%).ConclusionWe could demonstrate that 32% of patients remained relapse-free and 19% off glucocorticoids at 1 year after treatment with a rapid glucocorticoid tapering regimen. Most relapses were minor and could be handled with temporarily increased glucocorticoid doses. Consequently, the CSD at 12 months was much lower than reported in published cohorts. Thus, further reducing treatment-associated damage in patients with GCA by decreasing CSD seems to be possible.