OP0063 QUANTITATIVE COMPUTED TOMOGRAPHY PREDICTS 10-YEAR MORTALITY IN INTERSTITIAL LUNG DISEASE RELATED TO SYSTEMIC SCLEROSI

Background: Interstitial lung disease (ILD) is the main cause of death in Systemic Sclerosis (SSc). Chest CT is the gold standard in detecting ILD although it is not easy to understand its prognostic value. ILD qualitative assessment is almost worthless. Goh et al. semi quantitative score of ILD extent is related to mortality risk but it is burdened by relevant inter/intra-readers variability. An operator independent algorithm based on voxel-wise analysis proved to identify SSc patients with an increased risk of mortality according to prediction models. Objectives: To verify if quantitative analysis of chest CT (QCT) predict 10 years-mortality in SSc patients. Methods: SSc patients with availability of a chest CT were enrolled in 13 different centers. The CT voxel-wise analysis with a free software (www.horosproject.com) provided QCT indexes: kurtosis, skewness, mean lung attenuation and standard deviation. Patients

Efficacy and Drug Survival after Switching from Etanercept to the Biosimilar SB4: A Real-Life Long-Term Study

We evaluated the 3-year drug survival and efficacy of the biosimilar SB4/Benepali in rheumatoid arthritis (RA), psoriatic arthritis (PsA) and ankylosing spondylitis (AS) patients, pre-viously treated with etanercept (ETA). Drug survival rate was calculated using the Kaplan\u2013Meier method and Cox proportional hazard models were developed to examine predictors of SB4 discontin-uation. 236 patients (120 RA, 80 PsA and 36 AS), aged 60.7 \ub1 13.8 years and with an ETA duration of 4.1 \ub1 3.4 years were included. The 3-year retention rate for SB4 was 94.4%, 88% and 86% in AS, RA and PsA patients, respectively, with no difference between groups. Patients without comorbid disease had higher retention rates vs. patients with comorbid disease (90% vs. 60%, p < 0.0001). Disease activity, as measured by DAS28, DAPSA and BASDAI remained stable over the 3 years. Comorbid disease (hazard ratio; HR: 4.06, p < 0.0001) and HAQ at baseline (HR: 2.42, p = 0.0024) significantly increased the risk of SB4 discontinuation, while previous ETA duration was negatively associated with SB4 discontinuation (HR: 0.97, p = 0.0064). Forty-one (17.4%) patients left the study due to the interruption of the SB4 treatment, 31 (75.6%) discontinued due to inefficacy and 10 (24.4%) due to adverse events. This real-life study confirms the similar efficacy profile of ETA with long-term retention and a good safety profile in inflammatory arthritis patients

Central pontine and extrapontine myelinolysis despite careful correction of hyponatremia: clinical and neuropathological findings of a case

We report clinical, radiological and pathological findings in a patient with central pontine and extrapontine myelinolysis. The patient was a 61-year-old woman who had a radical mastectomy for breast cancer. Based on clinical evidence, acute hyponatremia had set in only a few days before onset of symptoms. The patient's disease progressed in two stages and became more severe during slow hyponatremia correction after 9 days from onset. Diffusion MRI provided early evidence of neurological lesions. In spite of a therapeutic attempt, the patient died unexpectedly 18 days after onset of her neurological disease due to massive pulmonary embolism. Histologically, our findings confirmed that the major features of central pontine myelinolysis in the acute stage are demyelination, the presence of large amounts of macrophages with no lymphocytic inflammatory reaction, and moderate astrocytosis. It is interesting to note that a monotypic immunological reaction persisted 19 days after radiological demonstration of parenchymal alterations

PS164 SYSTEMIC SCLEROSIS INTERSTITIAL LUNG DISEASE EVALUATION: COMPARISON BETWEEN SEMIQUANTITATIVE AND QUANTITATIVE CT ASSESSMENTS

Background: Pulmonary fibrosis is the main death cause in systemic sclerosis (SSc). Lung involvement is assessed with spirometry (which evaluates pulmonary function) and chest Computed Tomography (CT) scan (that identifies interstitial fibrosis). FVC < 70 % and DLco < 75 % are considered pulmonary functional values associated with a remarkable interstitial lung disease (ILD) and, therefore, they are adverse prognostical indicators. Both qualitative and semiquantitative radiologic ILD assessment have a considerable interobserver variability. To overcome this problem quantitative scores (called CT indexes) correlating with ILD extent detectable on chest CT have been proposed. The aims of this work are to find: a) whether there is a correlation between pulmonary functional tests and CT indexes , b) which CT indexes have the best performance in discriminating patients with a pulmonary function indicative of an extensive ILD. Methods: Chest TC and spirometry of 90 SSc patients (31 from Parma, 30 from Turin, 29 from Piacenza) meeting ACR criteria were performed. Digital Imaging and COmmunications in Medicine (DICOM) images of chest CT were processed with OsiriX (a free and user-friendly DICOM-viewer) in order to obtain patients' CT indexes. The Spearman rank test was used to verify the correlations between CT indexes and spirometrical measures. CT indexes discriminative ability was verified using ROC analysis. A p-value < 0.05 was considered statistically significant. Results: Whole lung kurtosis (tKurt) is the best FVC correlating CT index (rho = 0,623; p < 0,0001). Parenchymal lung skewness (nSkew) is the best CT index correlating with DLco (rho = -0,582; p < 0,0001). ROC analysis showed that tKurt = 6,32 can discriminate very well patients with FVC < 70% (sensibility 80,0%, specificity 74,3%). Similarly nSkew = 2,2 distiguishes subjects with DLco < 75% (sensibility 85,7%, specificity 52,2%). Conclusions: Spirometry and CT indexes correlations are consistent with literature. The identification of CT index values corresponding to spirometric cutoff indicative of a considerable limitation of lung function makes TC ILD quantification useful in establishing SSc patients prognosis. Obtaining CT indexes with a free and user-friendly software can contribute to widespread in clinical practice this new SSc ILD assessment

THU0616-HPR EXPIRATORY FLOW ACCELERATOR (EFA) IN SYSTEMIC SCLEROSIS PATIENTS WITH MUCUS HYPERSECRETION, PRODUCTIVE COUGH AND DYSPNOEA: PRELIMINARY RESULTS FROM A HOME-BASED AIRWAY CLEARANCE TECHNIQUE DAILY PROGRAM

Background:Systemic sclerosis (SSc) is a chronic disease with frequent lung involvement. As mucociliary clearance is impaired, mucus retention and frequent pulmonary infections, increase morbidity and mortality (1).Airway clearance techniques (ACT) enhance removal of mucus from the airways. Expiratory flow accelerator (EFA) is a new technology that promotes deep and gentle drainage of the bronchial secretions, through the Venturi effect. No respiratory effort is required and no negative pressure is generated, avoiding risk of bronchial collapse (2).Objectives:The aim of this study was to describe the effectiveness of EFA in improving pulmonary symptoms in SSc patients.Methods:SSc patients with daily productive cough, frequent pulmonary exacerbations, exertional dyspnea and/or reduced physical activity were selected. All of them underwent a home-based ACT program with EFA. A Respiratory Physiotherapist (RT) trained each patient to use the device 3 times a day, 15 minutes each session. Every subject compiled the Saint George's Respiratory Questionnaire (SGRQ) and scleroderma Health Assessment Questionnaire (SHAQ) at baseline, 30, 90 and 180 days from the beginning. Statistical analysis has been carried out with General linear model for repeated measures. A value of p<0.05 was considered statistically significant.Results:8 patients were enrolled (M:F=1:7), median age 54 (IC95% 46-69) years. Interstitial lung disease affected the majority of them (7/8). SGRQ total score and SHAQ domain for respiratory symptoms decreased over time (p= 0.003 and p= 0.005). In particular, there was an improvement in two SGRQ domains: activities (p= 0.013) and symptoms (p= 0.005) (fig.1).Figure 1Conclusion:This is the first study to investigate the effect of EFA technology on airway clearance in SSc patients. The observations suggest the importance of a daily ACT program with EFA in improving respiratory symptoms. This technology appear to be extremely promising in SS patient management as it is well tolerated and it has the potential to slow down the pulmonary disease progression by limiting bronchial infections.References:[1]Tyndall AJ et al. Causes and risk factors for death in systemic sclerosis: a study from the EULAR Scleroderma Trials and Research (EUSTAR) database. Ann Rheum Dis 2010;69:1809–15.[2]Zampogna E, et al. Expiratory Flow Accelerator (EFA) technique on mucus hypersecretion of COPD patients with reduced cough efficiency after a severe exacerbation. Int Clin Med 2019;3:1-6.Disclosure of Interests:Silvia faverzani: None declared, Andrea Becciolini Speakers bureau: Sanofi-Genzyme, UCB and AbbVie, ernesto crisafulli: None declared, filippo nocera: None declared, eleonora di donato: None declared, Flavio Mozzani: None declared, michele riva: None declared, Daniele Santilli: None declared, lorenza monica: None declared, annalisa barbieri: None declared, lissette barone: None declared, maurizio marvisi: None declared, veronica alfieri: None declared, annalisa frizzelli: None declared, Alfredo Chetta: None declared, ALARICO ARIANI: None declare

Identifying the symptom and functional domains in patients with fibromyalgia: results of a cross-sectional Internet-based survey in Italy

Fausto Salaffi,1 Flavio Mozzani,2 Antonella Draghessi,1 Fabiola Atzeni,3 Rosita Catellani,2 Alessandro Ciapetti,4 Marco Di Carlo,1 Piercarlo Sarzi-Puttini5 1Rheumatology Department, Polytechnic University of Marche, Jesi (Ancona), 2Department of Internal Medicine and Rheumatology, University Hospital of Parma, Parma, 3IRCCS Galeazzi Orthopedic Department, Milan, Italy; 4Rheumatology Department, Betsi Cadwaladr University Health Board, Glan Clwyd Hospital, Bodelwyddan, Denbighshire, Wales; 5Rheumatology Department, L. Sacco University Hospital, Milan, Italy Objective: The aims of this cross-sectional study were to investigate the usefulness of using an Internet survey of patients with fibromyalgia in order to obtain information concerning symptoms and functionality and identify clusters of clinical features that can distinguish patient subsets. Methods: An Internet website has been used to collect data. Fibromyalgia Impact Questionnaire Revised version, self-administered Fibromyalgia Activity Score, and Self-Administered Pain Scale were used as questionnaires. Hierarchical agglomerative clustering was applied to the data obtained in order to identify symptoms and functional-based subgroups. Results: Three hundred and fifty-three patients completed the study (85.3% women). The highest scored items were those related to sleep quality, fatigue/energy, pain, stiffness, degree of tenderness, balance problems, and environmental sensitivity. A high proportion of patients reported pain in the neck (81.4%), upper back (70.1%), and lower back (83.2%). A three-cluster solution best fitted the data. The variables were significantly different (P&lt;0.0001) among the three clusters: cluster 1 (117 patients) reflected the lowest average scores across all symptoms, cluster 3 (116 patients) the highest scores, and cluster 2 (120 patients) captured moderate symptom levels, with low depression and anxiety. Conclusion: Three subgroups of fibromyalgia samples in a large cohort of patients have been identified by using an Internet survey. This approach could provide rationale to support the study of individualized clinical evaluation and may be used to identify optimal treatment strategies. Keywords: fibromyalgia, Internet, FIQR, FAS, cluster analysis, SAPS, pai

Identifying the symptom and functional domains in patients with fibromyalgia: results of a cross-sectional Internet-based survey in Italy

An Internet website has been used to collect data. Fibromyalgia Impact Questionnaire Revised version, self-administered Fibromyalgia Activity Score, and Self-Administered Pain Scale were used as questionnaires. Hierarchical agglomerative clustering was applied to the data obtained in order to identify symptoms and functional-based subgroups. Three hundred and fifty-three patients completed the study (85.3% women). The highest scored items were those related to sleep quality, fatigue/energy, pain, stiffness, degree of tenderness, balance problems, and environmental sensitivity. A high proportion of patients reported pain in the neck (81.4%), upper back (70.1%), and lower back (83.2%). A three-cluster solution best fitted the data. The variables were significantly different (P<0.0001) among the three clusters: cluster 1 (117 patients) reflected the lowest average scores across all symptoms, cluster 3 (116 patients) the highest scores, and cluster 2 (120 patients) captured moderate symptom levels, with low depression and anxiety. Three subgroups of fibromyalgia samples in a large cohort of patients have been identified by using an Internet survey. This approach could provide rationale to support the study of individualized clinical evaluation and may be used to identify optimal treatment strategies

Psychometric properties of an index of three patient-reported outcome (PRO) measures, termed the CLinical ARthritis Activity (PRO-CLARA) in patients with rheumatoid arthritis. The NEW INDICES study

Objective To evaluate the psychometric properties of an index based on 3 patient-reported outcome measures, termed PRO-CLinical ARthritis Activity (PRO-CLARA), in order to facilitate rapid and easy rheumatoid arthritis (RA) activity assessment in daily routine. Methods 196 patients partially or not responding to disease modifying anti-rheumatic drugs (DMARDs) consented to participate in a multicentre cross-sectional study. For the evaluation of the psychometric properties of the PRO-CLARA, this population has been compared to another cohort of 247 outpatients with RA who were participating in a long-term observational study and who satisfying minimal disease activity and remission definitions. All patients completed the PRO-CLARA, combining patient's physical function, self-administered tender joint count and perception of global health status into a single measure of disease activity. Additional comparator composite indices were analysed. Internal consistency was assessed with Cronbach's alpha coefficient. A confirmatory factor analysis was carried out to test factor structure. Concurrent validity was analysed using Spearman's correlations and cross-tabulations. Discriminant validity to distinguish patients with active and non-active disease was assessed with receiver operating characteristic (ROC) curve analysis. For agreement analysis, kappa statistics were calculated. Results In testing for internal consistency, we found that Cronbach's alpha for the PRO-CLARA was 0.893, indicating high reliability. PRO-CLARA proved to be significantly correlated to established RA activity assessment tools. The area under ROC curve of the PRO-CLARA gives identical results to those provided by other comparator indices. Conclusion The study showed satisfactory psychometric properties of the PRO-CLARA