J. Canas da Silva

Assessment of salivary gland function in Sjögren's syndrome: The role of salivary gland scintigraphy

Salivary gland scintigraphy (SGS) is a non invasive method of salivary gland function assessment. This technique is easy to perform, reproducible and well tolerated by patients. Additionally, an abnormal salivary gland scintigraphy result is accepted by the American-European consensus group as a criterion for the diagnosis of Sjögrens syndrome. Scintigraphic evaluation of salivary gland function also plays an important role in therapeutic decision and patient follow-up. Schalls categorical classification is usually considered the standard method for salivary scintigraphy interpretation, though subjective and with limited capacity to discriminate borderline results. In order to improve the diagnostic accuracy of SGS, there has been an increasing interest in the quantification of glandular function. However, the debate on the most reliable and suitable parameters for the diagnosis of SS persists.

Characterization of damage in Portuguese lupus patients: analysis of a national lupus registry.

Background: Although the survival rate has considerably improved, many patients with systemic lupus erythematosus (SLE) develop irreversible organ damage. Objectives: The objectives of this paper are to characterize cumulative damage in SLE patients and identify variables associated with its presence and severity. Methods: A cross-sectional analysis of SLE patients from the Portuguese Lupus register Reuma.pt/SLE in whom damage assessment using the SLICC/ACR-Disability Index (SDI) was available was performed. Predictor factors for damage, defined as SDI ≥ 1, were determined by logistic regression analyses. A sub-analysis of patients with severe damage (SDI ≥ 3) was also performed. Results: In total, 976 patients were included. SDI was ≥1 in 365 patients, of whom 89 had severe damage. Musculoskeletal (24.4%), neuropsychiatric (24.1%) and ocular (17.2%) domains were the most commonly affected. Older age, longer disease duration, renal involvement, presence of antiphospholipid antibodies and current therapy with steroids were independently associated with SDI ≥ 1. The subpopulation with severe damage had, in addition, a greater interval between the first manifestation attributable to SLE and the clinical diagnosis as well as and more frequently early retirement due to SLE. Conclusions: This large lupus cohort confirmed that demographic and clinical characteristics as well as medication are independently associated with damage. Additionally, premature retirement occurs more often in patients with SDI ≥ 3. Diagnosis delay might contribute to damage accrual.

Effect of Comedication With Conventional Synthetic Disease-Modifying Antirheumatic Drugs on Retention of Tumor Necrosis Factor Inhibitors in Patients With Spondyloarthritis: A Prospective Cohort Study.

To evaluate whether use of comedication with conventional synthetic disease‐modifying antirheumatic drugs (csDMARDs) influences the retention of tumor necrosis factor inhibitors (TNFi) in patients with spondyloarthritis (SpA).

AB0581 Two years existence of reuma.pt/vasculitis – the portuguese registry of vasculitis

Background The vasculitides are a group of relatively rare diseases with different manifestations and outcomes. New therapeutic options have led to the need for long-term registries. The Rheumatic Diseases Portuguese Register, Reuma.pt, is an electronic clinical record, created in 2008, which currently includes specific protocols for 11 diseases and >16000 patients registered from 79 national and international rheumatology centres. Since October 2014, a dedicated protocol to vasculitis has been created as part of the European Vasculitis Society initiative of having compatible European registries. Objectives To describe the structure of Reuma.pt/Vasculitis and characterize the patients registered over the last two years. Methods We developed a dedicated web-based software to enable prospective collection and central storage of anonymised data from patients with vasculitis. All Portuguese rheumatology centres were invited to participate. Data regarding demographics, diagnosis, classification criteria, imaging and laboratory tests, outcome measures of prognosis, damage, disease activity and quality of life, and treatment were collected. We performed a cross-sectional descriptive analysis of all patients registered up to January 2017. Results A total of 492 patients, with 1114 visits, from 11 centres were registered in Reuma.pt/Vasculitis. The mean age was 53±20 years at last visit; 68% were females. The diagnoses followed the 2012 Chapel Hill Consensus nomenclature (Table 1). The most common diagnoses were Behçets disease (BD) (39%) and giant cell arteritis (GCA) (20%). Patients with BD met the International Study Group 1990 criteria, the International Criteria for BD 2006 and 2013 in 84%, 95% and 95% of cases, respectively. Patients with GCA met the 1990 American College of Rheumatology criteria in 95% of cases. Data on vascular ultrasound was available in 74% of patients; 73% compatible with the diagnosis. Assessment of the Birmingham Vasculitis Activity Score (BVAS) and Vasculitis Damage Index (VDI) was available for all vasculitides and the Five Factor Score calculation of survival rate for ANCA associated vasculitis (AAV) and polyarteritis nodosa (PAN). The mean BVAS at first visit was 18±7 for AAV and 15±9 for PAN; the mean VDI at last visit was 3±2 for AAV and 2±2 for PAN. Health related quality of life assessments (SF-36, EQD5, FACIT and HADS) were also collected. Treatment registry with the disease assessment variables shown in graphics was available for all patients; only 6% were under biologic treatment. Conclusions Reuma.pt/Vasculitis is a registry adapted for routine care, allowing an efficient data repository at a national level with the potential to link with other international databases. It facilitates research, trials recruitment, service planning and benchmarking. Disclosure of Interest None declared

THU0689 Moderate to good construct validity of global presenteeism measures with multi-item presenteeism measure and patient reported health outcomes: eular-pro worker productivity study

Background Inflammatory arthritis (IA) and osteoarthritis (OA) often impact on the workers performance whilst at work due to ill health (i.e. presenteeism). A number of global measures have been developed to assess presenteeism in clinical studies. However, limited information is available on the correlation between these measures and the construct validity of these measures. Objectives To i) determine the correlation between four global measures of presenteeism and ii) to evaluate the construct validity of these measures. Methods The main aim of this international observational study (7 countries in Europe and Canada), recruiting patients with IA (RA, PsA or AS) or OA in paid employment, was to investigate content and construct validity of global presenteeism measures. Patients completed 4 global measures (Work Productivity Scale – Arthritis (WPS-A), Work Productivity and Activity Impairment Questionnaire (WPAI), Work Ability Index (WAI), and both the Quality and Quantity scales of the QQ questionnaire) (see table legend for descriptions individual scales). Spearman correlations were applied to test the correlation between individual presenteeism scales and to test construct validity with the 11-item Workplace Activity Limitation presenteeism Scale (WALS) and several health related patient reported outcome measures. Interpretation of correlation coefficients: (very) weak (rrange=0.0–0.39), moderate (rrange=0.40–0.59) to strong (rrange=0.60–1.0). Results 468 patients with a median disease duration of 10 [IQR 5–18] yrs were included; 62% were female. Median [IQR] presenteeism scores were, respectively: 2 [0–5] for WPS-A, 3 [1–5] for WPAI, 8 [6–9] for WAI, 81 [49–100] QQ-total, with WAI and QQ having reversed scales (Legend table). Correlations between the 4 global measures were moderate to strong, ranging from -0.49 for the correlation between WPS-RA and QQ-Quality to 0.83 between WPAI and WPS-RA. The multi-item presenteeism scale WALS, measuring difficulty at work, strongly correlated with both WPAI (r=0.65, p<0.05) and WPS-RA (r=0.64, p<0.05), both measures capturing the affect and interference of arthritis on work productivity. Moderate correlations were achieved between all six global presenteeism measures and health outcomes (rrange irrespective direction: |rpresenteeism-VAS Well-being| =0.36 to 0.54; |rpresenteeism-EQ-5D| =0.37 to 0.54; and |rpresenteeism-HAQ| =0.40 to 0.58). Conclusions Global measures of presenteeism show good to moderate construct validity, with the WPAI and WPS-IA/OA showing slightly better construct validity compared to the WAI and QQ. The information obtained in this study will further inform research on instrument use for a standardized approach to estimate presenteeism in future clinical studies. Acknowledgements Funding: EULAR, AbbVie, BMS Disclosure of Interest None declared

AB1023 Rheuma Space: Standard Practice Aiming Clinical Excellence in Rheumatology

Background The evaluation of the quality of medical practice and the implementation of corresponding measures to improve it are crucial steps for the development of Rheumatology in Europe. Herein we describe the implementation of a national program, Rheuma Space, aiming at quality improvement in the field of Rheumatology. Objectives To develop standards for the quality of care accepted by most of the rheumatology services in Portugal. Methods Quality indicators were obtained through a four-step RAND-modified Delphi methodology. The first step involved a literature search for international benchmarking of quality of care initiatives and indicators, followed by a pre-selection of a initial set of criteria by a task force. The final steps, aiming at defining a smaller set of criteria that could best analyze rheumatology quality of care, encompassed an online Delphi round with all Portuguese rheumatologists and a consensus meeting with a panel of invited experts representing all the Portuguese Rheumatology Departments of the National Health Service. Results A total of 412 different indicators were collected throughout the first project phase and the final set of 26 quality indicators was defined, within the three Donabedian dimensions of healthcare quality: nine “structure”, eleven “processes” and seven “outcome” indicators. These criteria cover eleven domains of quality of care: personnel and organizational structure, training and research, facilities, equipment and IT, budgeting and financial resources, access to care, clinical records, patient communication, multidisciplinary management, clinical outcomes, and patient and personnel satisfaction. Conclusions The 26 quality indicators set constitutes the basis for a quality management tool, which is now being implemented in all the Portuguese Rheumatology Departments of the National Health Service. Direct surveys derived from the 26 quality indicators are being applied to health professionals and patients allowing to benchmark departments and to identify strengths and weakness for future improvement. This initiative is deemed to improve the process of care for Portuguese rheumatic patients, thereby ensuring quality standards of structure and process criteria, for a patient-oriented clinical practice, favouring desirable continuous quality improvement on health outcomes. Acknowledgement Portuguese Rheumatology participating in the online Delphi and all the participants in the expert consensus meeting Disclosure of Interest None declared

AB0636 Interstitial Lung Disease in Scleroderma Portuguese Patients: Table 1.

Background Systemic sclerosis (SSc) is a rheumatic disorder characterized by inflammation and fibrosis involving the skin as well as internal organs, including the lung. Objectives This study was conducted to characterize SSc patients with ILD and determine predictor features for the presence of ILD. Methods A retrospective evaluation of SSc patients from our Rheumatology department was undertaken. ILD was defined according to imaging findings in high-resolution CT (HRCT) combined with functional parameters. Demographic features, auto-antibodies, spirometry, diffusion capacity for carbon monoxide (DLCO) measurement, echocardiography and 6-minute walk test were compared between groups with and without ILD using parametric tests and non-parametric tests. Predictor factors were established by logistic regression analyses. Results One hundred and three SSc patients with current mean age of 60.2±14.1y and mean disease duration of 10.0±9.6y were evaluated. Thirty-four (35.8%) patients were diagnosed with ILD, of whom 13 had limited cutaneous SSc, 14 had diffuse cutaneous SSc, 2 sine sclerodema and 5 overlap syndroms. ANA was positive in 100% cases, anti-Scl70 was found in 55% and anti-centromere antibody positivity was found in 15% of patients. HRCT revealed diffuse parenchymal lung disease involvement >20% in 18 (53%) cases, with the majority showing a predominant honeycombing pattern. Spirometry showed a restrictive pattern in 8 patients and 1 had obstruction. The DLCOsb was abnormal in 7 patients. Three patients had pulmonary hypertension confirmed by right heart catheterization, 2 patients classified as group 1 and 3 and 1 patient of group 5. About 63% of ILD patients were ever treated with corticosteroids and 27% with cyclophosphamide, which were the most common drugs used. Two patients were also treated with rituximab. In this ILD-group, 5 deaths were recorded, 2 due to cancer, 1 abdominal sepsis, 1 pulmonary hypertension and 1 of unknown cause. Comparison of these ILD patients with non-ILD patients revealed some significantly differences (Table 1).Table 1. Statistically significant distinctions between patients with and without ILD ILD patients (n=34) No-ILD patients (n=61) P Current mean age (years) 61.8±14.5 58.6±13.2 0.330 Mean disease duration (years) 11.6±9.8 9.2±9.3 0.250 Anti-centromere antibody (%) 5 (15) 48 (79) <0.001 Anti-Scl70 antibody (%) 18 (55) 4 (7) <0.001 Diffuse cutaneous disease (%) 14 (41) 5 (8) <0.001 Digital ulcers (%) 19 (54) 11 (18) <0.001 NYHA Functional Classification (%) 23 (67) 30 (49) 0.017 Tricuspid regurgitation (%) 26 (84) 24 (45) 0.001 DLCOsb <60 (%) 7 (64) 0 (0) 0.056 Hypoxemia at rest (%) 8 (27) 4 (7) 0.013 Gastrointestinal involvement (%) 10 (48) 9 (24) 0.060 Death (%) 5 (16) 1 (2) 0.020 Multivariate analysis showed that digital ulcers (OR=31.6, 95% CI 3.09 to 321.91), Anti-Scl70 antibody positivity (OR=10.1, 95% CI 1.19 to 85.74) and anti-centromere antibody negativity (OR=0.08, 95% CI 0.01 to 0.83) were independently associated with the presence of ILD. Conclusions This study is one of the first studies carried out in Portugal regarding lung involvement in SSc. About one third of the patients had ILD, whose main characteristics are in accordance with other European cohorts. We confirmed that anti-SCL70 positivity, presence of digital ulcers as well as the absence of anti-centromere antibody is independently associated with ILD Disclosure of Interest None declared

OP0129 Nailfold Capillaroscopy in Rheumatology Practice - A Single Center Experience

Background Nailfold capillaroscopy (NCP) is an easy, non-invasive and useful tool for the diagnosis and follow-up of Systemic Sclerosis (SSc) and other systemic rheumatic diseases. Objectives To present our single center experience with NCP, describing the main reasons for NCP request, and the major abnormalities detected in capillaroscopy. Methods Retrospective analysis of patients submitted to NCP assessment, from January 2013 to December 2015 in our center. We collected reasons for NCP request, demographic and clinical data, autoimmunity profile and NCP results. Results A total of 221 NPC, corresponding to 197 patients, 167 females (84.8%) and 30 males (15.2%) aged from 9 to 90 years were reviewed. One hundred and thirty eight (62.4%) patients had no definitive diagnosis, 53 patients (24%) had SSc, 7 (3.2%) had mixed connective tissue disease, 3 (1.4%) had dermatomyositis/polymyositis (DM/PM), and 3 (1.4%) antiphospholipid syndrome (APS). Raynauds phenomenon (RP) alone was the most frequent reason for requesting NCP (33% of the cases), and combined with ANA positivity accounted for another 30.8% of the requests. RP was present in 73.3% of the patients, acrocyanosis in 16.3%, livedo reticularis and puffy hands accounted for 8.6% each one. Anti-nuclear antibodies (ANA) were positive in 59.7%, anti-centromere antibodies were present in 21.3%, anti-topoisomerase I and SSA/SSB in 5.1% of the cases. One hundred and fifty nine (71.9%) NCP were considered abnormal. The most frequent specific disease pattern was the early one (Cuttolo) in 15%. The most frequent findings were: capillary tortuosity in 112 (71.8%) cases, capillary dilatations in 95 (60.9%), and hemorrhages in 85 (54.5%). NCP was abnormal in 79.4% of patients with RP and ANA, 41.2% of them displaying specific findings of rheumatic diseases (Table 1). In SSc patients the late and the early pattern (Cuttolo) were the most frequent, present in 15 and 13 NCP, respectively. Concerning specific NCP findings capillary dilatations (81%), hemorrhages (73.6%) and megacapillaries (71.7%) were the most frequent (Table 2). Of the 138 cases without definitive diagnosis, 61. 6% had abnormal NCP, although mostly with non-specific patterns (50%). In 10 (7.2%) patients NCP helped to establish the diagnosis: VEDOSS (Very Early Diagnosis of Systemic Sclerosis) in 6, antiphospholipid syndrome in 3 and DM in one case. Conclusions In our experience, NCP was helpful for the diagnosis of rheumatic systemic disease. Specific NCP findings were more frequent in patients with RP + ANA positivity, suggesting that this combination is associated with more disease incidence. Patients with isolated RF had less pathologic NCP findings than those with isolated. ANA positivity. The prospective follow up of those patients with abnormal NCP and no definitive diagnosis will help to establish the predictive value of observed alterations. References Cutolo M, Pizzorni C, Sulli A. Capillaroscopy. Best Pract Res Clin Rheumatol. 2005 Jun;19(3):437–52 Disclosure of Interest None declared

SAT0237 Nailfold Capillaroscopy Findings in Scleroderma Patients – Prognostic Implications

Background Nailfold capillaroscopy (NCP) is a useful tool for the diagnosis and follow-up of systemic rheumatic diseases and represents one of the best methods to evaluate microvascular abnormalities. Objectives To characterize NCP findings of patients with Systemic Sclerosis (SSc), and understand how NCP associates with the presence of digital ulcers, gastrointestinal involvement, pulmonary arterial hypertension (PAH) and interstitial lung disease (ILD). Methods NCP findings of adult patients with SSc followed-up in our center were reviewed. Demographic and clinical features were collected. Nonparametric tests were used to determine potential associations between capillaroscopy findings/patterns and the presence of digital ulcers, gastrointestinal involvement, PAH and ILD. Results In total, 52 out of 103 patients with SSc had at least one NCP result available. Forty five were female (86.5%) and seven male (13.5%), the age was 56.6 ±13.2 years and disease duration 10.9 ±10.5 years. Thirty one (59.6%) had limited cutaneous SSc, seven (13.5%) diffuse cutaneous SSc, ten (19,2%) VEDOSS, three (5.8%) overlap syndromes and one (1.9%) SSc sine scleroderma. (table 1). The most frequent NCP findings were hemorrhages (54.7%) megacapillaries (54.7%), and capillary dilatations (52.8%). We found that digital ulcers were significantly associated with the existence of avascular areas (p=0.02), and with neoangiogenesis (p=0.03) in univariate analysis, but were not associated with any capillaroscopy pattern. Looking to interstitial lung disease, there is a trend for association with the presence of avascular areas (p=0.056). Only one patient had PAH confirmed by right heart catheterization. In this case NCP showed giant capillaries, neoangiogenesis and avascular areas. There was also a trend for association between avascular areas and higher values of NT pro BNP (p=0.078). Gastrointestinal involvement was not significantly associated with NCP findings (table 2).Table 1. Distribution of patients according to NCP and disease subtypes Diffuse cutaneous Limited cutaneous Overlap syndrome VEDOSS Sine scleroderma (n=7) (n=31) (n=3) (n=10) (n=1) Early pattern n (%) 0 (0) 9 (29) 1 (33) 6 (60) 0 (0) Active pattern n (%) 1 (14.2) 5 (16.1) 1 (33) 2 (20) 0 (0) Late pattern n (%) 3 (42.9) 7 (22.6) 0 (0) 0 (0) 0 (0) Nonspecific n (%) 3 (42.9) 8 (25.8) 1 (33) 1 (10) 1 (100) Normal NCP n (%) 0 (0) 2 (6.5) 0 (0) 1 (11) 0 (0) Conclusions In our study the presence of avascular areas and neoangiogenesis in NCP was significantly associated with the existence of digital ulcers. Also, ILD and higher values of NT-proBNP were more common in patients who present avascular areas. These results suggest that abnormalities detected in the NCP might help predict organ involvement, although long term follow up and greater numbers of patients are needed in order to confirm the prognostic value of NCP findings. References Mannarino E, Pasqualini L, Fedeli F, Scricciolo V, Innocente S. Nailfold capillaroscopy in the screening and diagnosis of Raynauds phenomenon. Angiology 1994; 45: 37–42 Disclosure of Interest None declared

AB0479 Treatment Persistence of Biological Therapy in Rheumatoid Arthritis (RA) Patients in Routine Clinical Practice

Background The use of biological DMARDs to treat RA patients has increased over the past 15 years probably due to their efficacy and tolerability profile. Objectives Describe persistence (time between biological agent initiation and its discontinuation), the impact of biological therapy (BT) in clinical outcomes (DAS28) and the reasons for its discontinuation. Methods A retrospective study of RA patients (pts) followed at Hospital Garcia de Orta with 1st BT [abatacept (ABT), adalimumab (ADA), anakinra (ANA), etanercept (ETN), golimumab (GLM), infliximab (IFX), rituximab (RTX), and tocilizumab (TCZ)] starting between 1/1/2001 and 31/7/2014. Electronic clinical records (Reuma.pt database) were reviewed for all pts that have been treated at least 12 months previously to their recruitment. Available demographic, clinical and therapeutic data were collected. Survival methods were used to analyze time to an event. Results 97 RA pts with mean age at RA diagnosis of 45.4±13.4 years were included. 86% were women, 53% with erosive disease, 66% with positivity for rheumatoid factor and 70% with positivity for anti-CCP antibodies. The mean age at the beginning of BT was 53.0±12.7y. Concerning 1st BT, ETN was the most frequent one (36%), followed by IFX (30%), and ADA (27%). 61 pts discontinued their 1st BT, mainly due to secondary ineffectiveness (15 IFX, 10 ETN, 9 ADA, 2 ANA and 1 TCZ). The median time on 1st line was 59.6 months (plot 1). The treatment persistence was not significantly different between BT (p=0.6). Notwithstanding, ETN showed a higher median persistence (96.1 months) than the others (plot 2). Comparing with IFX, the adjusted risk of discontinuation was 42% and 33% inferior with ETN and ADA respectively, though statistical significance was not reached. More than 50% of pts discontinued 1st BT within first 5 years. 46 pts started a 2nd BT (19 ETN, 13 ADA, 5 IFX, 5 RTX and 4 others). Of these, 21 discontinued most due to secondary ineffectiveness (7 ADA, 3 IFX and 3 ETN). The median time on 2nd line was 43.2 months (plot 3). The differences between BT in persistence are fairly statistically significant (p=0.06). Again, ETN showed a higher median persistence (43.5 months) followed by ADA (11.5 months) (plot 4). ADA as 2nd BT presented an adjusted risk to discontinue almost 4 times higher than ETN (HR=3.98, p=0.02). 15 pts switched their 2nd BT (5 TCZ, 4 ABT, 4 RTX e 2 ETN). More than 50% of the pts discontinued their 2nd BT within 4 years after starting it. The adjusted risk for switching with ADA was 9 times higher than the risk for switching ETN (HR=9.06, p=0.004). Regarding evolution of DAS28 during 1st line, ETN presented the highest variation from the baseline at 3, 6, 12, 18 and 30 months compared with all other agents. Regarding 2nd BT, the longitudinal analysis revealed that all agents achieved response between 6 and 18 months after treatment initiation. Conclusions The analysis per BT suggests that ETN was the TNF antagonist with the highest persistence on treatment and efficacy profile. Comparing 1st and 2nd lines of biological therapy, there is a decrease of 16.4 months on persistence. Additionally, the probability of switch increases and the probability of end of treatment decreases from 1st to 2nd line. Disclosure of Interest None declared