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Objective To develop and validate the prognostic prediction model DU-VASC to assist the clinicians in decision-making regarding the use of platelet inhibitors (PIs) for the management of digital ulcers in patients with systemic sclerosis. Secondly, to assess the incremental value of PIs as predictor. Methods We analysed patient data from the European Scleroderma Trials and Research group registry (one time point assessed). Three sets of derivation/validation cohorts were obtained from the original cohort. Using logistic regression, we developed a model for prediction of digital ulcers (DUs). C-Statistics and calibration plots were calculated to evaluate the prediction performance. Variable importance plots and the decrease in C-statistics were used to address the importance of the predictors. Results Of 3710 patients in the original cohort, 487 had DUs and 90 were exposed to PIs. For the DU-VASC model, which includes 27 predictors, we observed good calibration and discrimination in all cohorts (C-statistic = 81.1% [95% CI: 78.9%, 83.4%] for the derivation and 82.3% [95% CI: 779.3%, 85.3%] for the independent temporal validation cohort). Exposure to PIs was associated with absence of DUs and was the most important therapeutic predictor. Further important factors associated with absence of DUs were lower modified Rodnan skin score, anti-Scl-70 negativity and normal CRP. Conversely, the exposure to phosphodiesterase-5 inhibitor, prostacyclin analogues or endothelin receptor antagonists seemed to be associated with the occurrence of DUs. Nonetheless, previous DUs remains the most impactful predictor of DUs. Conclusion The DU-VASC model, with good calibration and discrimination ability, revealed that PI treatment was the most important therapy-related predictor associated with reduced DU occurrence.

ObjectivesPatient-reported outcome measures (PROMs) are important for clinical practice and research. Given the high unmet need, our aim was to develop a comprehensive PROM for systemic sclerosis (SSc), jointly with patient experts.MethodsThis European Alliance of Associations for Rheumatology (EULAR)-endorsed project involved 11 European SSc centres. Relevant health dimensions were chosen and prioritised by patients. The resulting Systemic Sclerosis Impact of Disease (ScleroID) questionnaire was subsequently weighted and validated by Outcome Measures in Rheumatology criteria in an observational cohort study, cross-sectionally and longitudinally. As comparators, SSc-Health Assessment Questionnaire (HAQ), EuroQol Five Dimensional (EQ-5D), Short Form-36 (SF-36) were included.ResultsInitially, 17 health dimensions were selected and prioritised. The top 10 health dimensions were selected for the ScleroID questionnaire. Importantly, Raynaud’s phenomenon, impaired hand function, pain and fatigue had the highest patient-reported disease impact. The validation cohort study included 472 patients with a baseline visit, from which 109 had a test–retest reliability visit and 113 had a follow-up visit (85% female, 38% diffuse SSc, mean age 58 years, mean disease duration 9 years). The total ScleroID score showed strong Pearson correlation coefficients with comparators (SSc-HAQ, 0.73; Patient’s global assessment, Visual Analogue Scale 0.77; HAQ-Disability Index, 0.62; SF-36 physical score, −0.62; each pConclusionsWe have developed and validated the EULAR ScleroID, which is a novel, brief, disease-specific, patient-derived, disease impact PROM, suitable for research and clinical use in SSc.

BackgroundThe gastrointestinal (GI) tract is frequently affected in systemic sclerosis (SSc), leading to considerable morbidity and even mortality. While important progress has been made in the last years regarding treatment of SSc, there is no disease-modifying treatment available for SSc-related GI involvement.ObjectivesWe aimed to identify, in an observational cohort study of real-life patients with SSc, an association between immunosuppressive therapy and the severity of GI symptoms, measured by the University of California at Los Angeles / Scleroderma Clinical Trial Consortium Gastro-Intestinal Tract instrument 2.0 (UCLA GIT 2.0).MethodsWe selected patients from our EUSTAR centre who met the 2013 ACR/EULAR classification criteria for SSc and had at least two visits with completed UCLA GIT 2.0 questionnaires, with an interval of 12±3 months between visits. We defined the first visit with a completed UCLA GIT 2.0 questionnaire as baseline visit. Immunosuppressive therapy was defined as exposure for at least 6 months between the two visits to at least one of the following drugs, regardless of indication: mycophenolate mofetil (MMF), cyclophosphamide, methotrexate, azathioprine, leflunomide, glucocorticoids (>10mg/d prednisone-equivalent), rituximab, tocilizumab, and abatacept. The study outcome was the UCLA GIT 2.0 score at the follow-up visit. We performed multivariable linear regression with this outcome as dependent variable and immunosuppressive therapy during follow-up, immunosuppressive therapy before baseline, baseline UCLA GIT 2.0 score and several baseline parameters selected by clinical judgment as potentially influencing GI symptoms, as independent variables. Multiple imputation was implemented to handle missing values.ResultsWe included 209 patients. Baseline characteristics were: 82.3% female, median (IQR) age 59.0 (48.6, 68.2) years, median disease duration 6.0 (2.7, 12.5) years, 40 (19.1%) diffuse cutaneous SSc, median baseline UCLA GIT 2.0 score 0.19 (0.06, 0.43). Of these, 71 patients were exposed to immunosuppressive therapy during the observation period: 27/71 methotrexate, 1/71 cyclophosphamide, 17/71 MMF, 3/71 leflunomide, 3/71 azathioprine, 6/71 glucocorticoids >10mg/d, 16/34 rituximab, 18/34 tocilizumab. Patients on immunosuppressive therapy during the observation period had, compared to patients without such treatment, overall more severe SSc, higher prevalence of treatment with proton pump inhibitors, similar UCLA GIT 2.0 scores at baseline and at follow up and tendentially less severe GI symptoms at baseline and follow-up by medical history. In multivariable linear regression, immunosuppressive therapy, lower body mass index, longer disease duration and lower baseline UCLA GIT 2.0 score were significantly associated with lower (better) UCLA GIT 2.0 scores at follow-up (Table 1).Table 1.Predictors of UCLA GIT 2.0 score at follow-upEstimates95% CIpAge0.002-0.001 – 0.0060.136Sex [male]-0.056-0.172 – 0.0610.347Disease duration-0.005-0.009 – -0.0000.030Body mass index0.0140.002 – 0.0250.017UCLA GIT 2.0 total score baseline0.6900.571 – 0.809Immunosuppressive therapy during observation period-0.119-0.228 – -0.0100.032Immunosuppressive therapy before baseline0.080-0.032 – 0.1920.160Modified Rodnan Skin Score-0.001-0.008 – 0.0070.860Forced vital capacity-0.001-0.004 – 0.0010.302Erythrocyte sedimentation rate0.003-0.001 – 0.0060.116Proton pump inhibitors-0.034-0.120 – 0.0520.435(Intercept)-0.120-0.531 – 0.2910.566Baseline factors associated with the total UCLA GIT 2.0 score at the end of the observation period. Multiple linear regression model with imputation for missing variables. N=209 patientsConclusionImmunosuppressive treatment was associated with lower UCLA GIT 2.0 scores, which suggests potential effects of immunosuppressants on GI manifestations in patients with SSc. These results need verification in additional studies and randomised controlled clinical trials.References[1]Khanna D et al. Arthritis Rheum, 2009; 61: 1257-63.Disclosure of InterestsLea Stamm: None declared, Alexandru Garaiman: None declared, Norina Zampatti: None declared, Mike O. Becker Speakers bureau: Mepha, MSD, Novartis, GSK, Bayer and Vifor, Consultant of: Mepha, MSD, Novartis, GSK, Bayer and Vifor, Grant/research support from: Mepha, MSD, Novartis, GSK, Bayer and Vifor, Cosimo Bruni Speakers bureau: Actelion, Eli-Lilly, Boehringer-Ingelheim, Grant/research support from: Abbvie, EUSTAR, Gruppo Italiano Lotta alla Sclerodermia (GILS), SCTC, Rucsandra Dobrota Consultant of: Boehringer-Ingelheim, Grant/research support from: Iten-Kohaut Foundation, Muriel Elhai: None declared, Sherif Ismail Grant/research support from: EULAR scientific training grant for young fellows 2021, Suzana Jordan: None declared, Aurora Tatu: None declared, Oliver Distler Speakers bureau: Bayer, Boehringer Ingelheim, Janssen, Medscape, Consultant of: Abbvie, Acceleron, Alcimed, Amgen, AnaMar, Arxx, AstraZeneca, Baecon, Blade, Bayer, Boehringer Ingelheim, Corbus, CSL Behring, 4P Science, Galapagos, Glenmark, Horizon, Inventiva, Kymera, Lupin, Miltenyi Biotec, Mitsubishi Tanabe, MSD, Novartis, Prometheus, Roivant, Sanofi and Topadur, Grant/research support from: Kymera, Mitsubishi Tanabe, Boehringer Ingelheim, Carina Mihai Speakers bureau: Boehringer-Ingelheim, Mepha, MED Talks Switzerland, Consultant of: Boehringer-Ingelheim, Janssen, Grant/research support from: Boehringer-Ingelheim, Janssen, Roche.

BackgroundUpper-gastrointestinal involvement (GI) is associated with more severe interstitial lung disease in patients with systemic sclerosis (SSc-ILD). However, there are many unexplored GI risk factors for the presence of SSc-ILD which could be potentially revealed by machine learning algorithms.ObjectivesThe aim of our study was to identify GI related risk factors for the presence of SSc-ILD using machine learning algorithms based on decision trees (DT).MethodsData of the last follow-up visit from consecutive patients fulfilling the 2013 ACR/EULAR SSc classification criteria recorded in our local EUSTAR registry were used for this study.The study outcome was the presence of SSc-ILD on high-resolution computed tomography.Two sets of predictors were identified based on their potential association with GI. The first set contains the following variables available in the EUSTAR registry: esophageal symptoms (dysphagia and reflux), stomach symptoms (early satiety, vomiting), intestinal symptoms (diarrhea, bloating and constipation), malabsorption syndrome, body-mass-index (BMI) and proton pump inhibitor therapy, calcium channel blocker therapy and immunosuppressive therapy. In the second set, we replaced the first three EUSTAR variables of the first set with the scales of the UCLA Gastrointestinal Tract Questionnaire 2.0 (UCLA-GIT).Of these two sets, the most important variable was selected using three different DT-based algorithms: (1) recursive partitioning and regression trees (RPART) –which uses trees to build decision rules, (2) random forest (RF) - an ensemble of DT built in parallel, and (3) gradient boosting machines (GBM) - an ensemble of DT built sequentially. The selected variables were eventually integrated with established predictors for presence of SSc-ILD (diffuse cutaneous subset, anti-Scl-70 positivity, male gender, forced vital capacity [FVC] and diffusion capacity of the lung for carbon monoxide-single breath [DLCO-SB]) into final prediction models for SSc-ILD presence using RPART, RF and GBM respectively. Their performance was evaluated by C-statistics. The importance of the newly detected predictor was assessed by variable importance plots (VIPs).ResultsWe included in our study 334 patients. The median age was 61 [IQR: 50-69] years, 59 (17.7%) were males and 266 (79.6%) had limited cutaneous SSc. Median BMI was 23 [IQR: 21-26] kg/m2, 133 (39.8%) of the patients had SSc-ILD, median FVC% 93 [IQR: 81-105], DLCO 72.5 [56-84] and. Of the UCLA-GIT scales the highest score was for the distension/bloating with a value of 0.50 [IQR: 0-1.24]. Regarding medications, 167 (50%) patients were exposed to PPI, 39 (11.7%) to CCB and 105 (31.4%) to immunosuppressive therapy.The BMI was deemed by all three algorithms as the most important predictor of SSc-ILD among both sets of GI related variables (Figure 1A-F). The final model, which included established risk factors for presence of ILD and the BMI, supported the importance of BMI in predicting the SSc-ILD. The VIPs obtained by GBM also ranked the BMI as the most important predictor.Figure 1.Tree-based algorithms revealing the importance of BMI for prediction of SSc-ILD. Panels A, B and C are variable importance plots (VIPs), which reveals the most important GI-predictor for occurrence of SSc-ILD in the EUSTAR set– the predictor with the highest relative importance is the most important predictor. Panels D, E and F are VIPs reveals the most important GI-predictor for occurrence of SSc-ILD in the UCLA-GIT set.A lower BMI was associated with presence of SSc-ILD (C-statistics for the RPART, RF and GBM models were 0.79, 0.70 and 0.76, respectively, corresponding to a fair accuracy). As expected, also a lower FVC, and DLCO-SB, and a positivity for Scl-70 ab were associated with presence of ILD.ConclusionLower BMI is a novel promising predictor for the presence of ILD, which should be confirmed in additional analyses.Disclosure of InterestsAlexandru Garaiman: None declared, Carina Mihai Speakers bureau: MEDtalks Switzerland, Mepha, Rucsandra Dobrota Consultant of: Actelion and Boehringer-Ingelheim, Grant/research support from: Articulum Fellowship, Pfizer, Actelion, Cosimo Bruni Speakers bureau: Eli-Lilly, Actelion, Boehringer-Ingelheim, Grant/research support from: Gruppo Italiano Lotta alla Sclerodermia (GILS), European,, Scleroderma Trials and Research Group (EUSTAR), Scleroderma Clinical Trials Consortium (SCTC), AbbVie, Muriel Elhai: None declared, Suzana Jordan: None declared, Lea Stamm: None declared, Anna-Maria Hoffmann-Vold Speakers bureau: Actelion, Boehringer Ingelheim, Jansen, Roche, Merck Sharp & Dohme, ARXX Therapeutics, Lilly and Medscape, Consultant of: Actelion, Boehringer Ingelheim, Jansen, Roche, Merck Sharp & Dohme, ARXX Therapeutics, Lilly and Medscape, Grant/research support from: Boehringer Ingelheim, Bayer, Oliver Distler Speakers bureau: Bayer, Boehringer Ingelheim, Medscape, Novartis, Roche, Pfizer, Roche, Sanofi, Consultant of: Abbvie, Acceleron, Alcimed, Amgen, AnaMar, Arxx, AstraZeneca, Baecon, Blade, Bayer, Boehringer Ingelheim, ChemomAb, Corbus, CSL Behring, Galapagos, Glenmark, GSK, Horizon, Inventiva, iQvia, Kymera, Lupin, Medac, Medscape, Miltenyi Biotec, Mitsubishi Tanabe, MSD, Prometheus Biosences, Roche, Roivant, Topadur and UBC, Lilly, Pfizer, Grant/research support from: Kymera, Mitsubishi Tanabe, Mike O. Becker Speakers bureau: Mepha, MSD, Novartis, GSK, Bayer and Vifor

BackgroundInterstitial lung disease (ILD) is the leading cause of death in systemic sclerosis (SSc). European consensus guidelines consider that some patients with mild disease might not need pharmacological treatment (1). Up to now, the disease characteristics and the disease course of non-treated SSc-ILD patients remain unknown.ObjectivesTo describe disease characteristics and the disease course of non-treated SSc patients with ILD.MethodsWe included patients from our local EUSTAR center registered since 2008, who had a diagnosis of ILD on high-resolution computed tomography (HRCT) and available data on pulmonary function tests and treatment. Longitudinal study included patients with at least one follow-up visit. Patients were classified as treated if they received a potential ILD modifying drug (immunosuppressive therapy or nintedanib). Treated and untreated patients were compared at baseline. Progression in the untreated group was defined as (i) forced vital capacity (FVC) decline from baseline of ≥10% or (ii) an FVC decline of 5-9% in association with a decline in diffusing capacity for carbon monoxide (DLCO) of ≥15%, or (iii) start of a ILD modifying treatment during follow-up. In the untreated group, patients who progressed at any time were compared with patients with stable disease during follow-up. Multivariable logistic regression was performed to identify (i) factors associated with non-prescription of a treatment in ILD patients at baseline and (ii) factors associated with progression in the untreated patients. Covariates were selected according to clinical experience and literature evidence.ResultsAmong 496 patients included in our cohort, 209 (42%) patients had ILD on baseline HRCT: 48/209 (23%) were males, median disease duration 8 [IQR: 4-12] years, 67/209 (32%) of diffuse cutaneous subset and 86/209 (41%) had anti-Scl70 antibodies.Among them, 142/209 (68%) did not receive any potentially ILD modifying treatments at baseline. Untreated patients were older (59 vs. 54 years), had a longer disease duration, were less frequently smokers, had more frequently anticentromere antibodies and lower levels of CRP. They had more frequently a limited extent (In multivariable logistic regression, older age (OR: 1.04 [1.01-1.08], p=0.021), a less extensive disease on HRCT (OR: 0.29 [0.09-0.90], p=0.037) and less frequent prescription of glucocorticoids (OR: 0.036 [0.12-0.92], p=0.037) were independently associated with absence of ILD modifying treatment prescription in our cohort.From the 142 untreated patients, 96 were followed-up for 64 [39-96] months. Of these, 56 (58%) patients showed progression of ILD, of whom 43 progressed by lung function parameters. Of these 56 patients, 31 (56%) progressed in the first 18 months. Diffuse cutaneous subtype (OR: 5.26 [1.26-27.62], p=0.031), shorter disease duration (OR: 0.95 [0.90-0.99], p=0.035) and oesophageal symptoms (reflux, dysphagia) (OR: 3.51 [1.12-12.18], p=0.036) at baseline were independent predictors of progression during follow-up in untreated patients.ConclusionA considerable number of SSc patients with ILD are not treated in clinical practice, in particular patients with limited cutaneous SSc, older age and an overall less extensive ILD. However, during a follow-up of 5 years, contrary to the common belief, about 60% of the untreated patients showed ILD-progression. The diffuse cutaneous subtype, shorter disease duration and oesophageal symptoms at baseline characterized these patients. With the development of effective and safe therapies for SSc-ILD, our results support a change in practice for selecting patients for treatment.References[1]Hoffmann-Vold A-M, et al. The Lancet Rheumatology. 2020;2(2):e71-e83.Disclosure of InterestsMoritz Scheidegger: None declared, Alexandru Garaiman: None declared, Carina Mihai Speakers bureau: Boehringer-Ingelheim, MED Talks Switzerland, Consultant of: Boehringer-Ingelheim (advisory board), Janssen (advisory board), Mike O. Becker Speakers bureau: Mepha, MSD, Novartis, GSK, Bayer and Vifor, Consultant of: Mepha, MSD, Novartis, GSK, Bayer and Vifor (advisory board fees), Rucsandra Dobrota Consultant of: Boehringer-Ingelheim (Advisory Board), Cosimo Bruni Speakers bureau: Eli-Lilly2018-2021, Actelion2019, Boehringer-Ingelheim2020-2021, Grant/research support from: AbbVie (educational grant 2021), Suzana Jordan: None declared, Håvard Fretheim Speakers bureau: Personal fees form Bayer and non-financial support from GSK and Actelion, outside the submitted work., Øyvind Midtvedt: None declared, Hilde Jenssen Bjørkekjær: None declared, Imon Barua: None declared, Anna-Maria Hoffmann-Vold Speakers bureau: Actelion, Boehringer Ingelheim, Jansen, Lilly, Medscape, Merck Sharp & Dohme, Roche, Consultant of: Actelion, ARXX, Bayer, Boehringer Ingelheim, Jansen, Lilly, Medscape, Merck Sharp & Dohme, Roche, Grant/research support from: Boehringer Ingelheim, Oliver Distler Speakers bureau: Bayer, Boehringer Ingelheim, Janssen, Medscape, Consultant of: Abbvie, Acceleron, Alcimed, Amgen, AnaMar, Arxx, AstraZeneca, Baecon, Blade, Bayer, Boehringer Ingelheim, Corbus, CSL Behring, 4P Science, Galapagos, Glenmark, Horizon, Inventiva, Kymera, Lupin, Miltenyi Biotec, Mitsubishi Tanabe, MSD, Novartis, Prometheus, Roivant, Sanofi and Topadur, Grant/research support from: Kymera, Mitsubishi Tanabe, Boehringer Ingelheim, Muriel Elhai Speakers bureau: Speaker fees: BMS outside the submitted work

BackgroundAn accurate assessment of nailfold capillaroscopy (NFC) images has great importance in the diagnosis and prognosis of systemic sclerosis (SSc). To overcome some of the inherent problems with NFC image analysis (operator/observer bias, time requirements), there is an interest to automate and standardize NFC image assessment using computer vision algorithms, such as the Vision Transformer (ViT), which are based on recent advances in deep learning.ObjectivesOur aims were (1) to implement and assess the performance and the reliability of ViT in detecting changes on NFC images, and (2) to compare the performance of ViT to that of practicing rheumatologists.MethodsFor this study, we used NFC images of patients with SSc enrolled in our European Scleroderma Trials and Research group (EUSTAR) and Very Early Diagnosis of Systemic Sclerosis (VEDOSS) local registries. Concretely, we included routine NFC images (all NFC images available – digit II to V of both hands-, irrespective of any image artefacts) of patients aged ≥ 18 years with visits between 2012 and 2021, who fulfilled either the 2013 American College of Rheumatology / European League Against Rheumatism classification criteria for SSc (established disease) or the preliminary criteria for VEDOSS (mild/early disease).ViT was trained to identify the following NFC signs of microangiopathy: enlarged capillaries (apex diameter of the capillary > 20 µm, and < 50µm), giant capillaries (> 50 µm), loss of capillaries (< 7 capillaries/mm), and microhaemorrhages. Absence of any of these signs was considered as being a “normal NFC”, while presence of any of these signs was seen as an abnormal NFC image. Its performance was evaluated in a cross-fold (k = 5) validation setting, and the gold standard was the treating physician. Area under the ROC curve (AUC) was used as indicator of the performance. Considered cut-offs for AUC were 90-100% - excellent, 80-89.9% - good, 70-79.9% - fair, 60-69.9% - poor, 50-59.9% - fail. From the first cross-fold, a randomly sampled reliability set was created which was used to compare the NFC assessment performance of four rheumatologists (three invited and trained annotators and the treating physician) with that of of ViT.ResultsWe analysed 17,126 NFC images of 234 EUSTAR patients (14.6% males, 67.8% limited cutaneous SSc, median age 57 years, median disease duration 9 years) and 55 VEDOSS patients (92.7% females, median age 44 years, median time elapsed since first Raynaud’s phenomenon 5 years). ViT had fair to excellent performance in identifying the different NFC changes across all five folds (of 3443 NFC images each), with an area under the ROC curve ranging from 78.59% to 90.4% (Figure 1a).Figure 1.Performance of the ViT: (a) AUCs values for ViT in diagnosing the respective feature on NFC image. (b) Sensitivity / specificity trade-off across the images in the reliability set. The blue lines show the ROC curves corresponding to the ViT predictions, with ViT’s AUC, “optimal” sensitivity and specificity shown in the box. Also shown are the point performances of each of the four annotators, with performances below the blue line indicating lower performance than ViT.In the reliability set (see Figure 1b), we observed highest performance for diagnosing giant capillaries (AUC =92.89%) followed by identification of enlarged capillaries (AUC = 91.7%). Good AUCs were seen in depicting capillary loss (AUC = 87.3%), microhemorrhages (AUC = 85.9%) and the abnormal/normal NFC classification (AUC = 84.7%). The rheumatologists had generally higher performance in assessing NFC images. However, ViT outperformed two rheumatologists with different experience in classifying capillary loss and enlarged capillaries, respectively.ConclusionViT is a modern, well performing and readily available AI model to assess signs of microangiopathy on NFC images acquired during routine practice.Disclosure of InterestsAlexandru Garaiman: None declared, Farhad Nooralahzadeh: None declared, Carina Mihai Speakers bureau: MEDtalks Switzerland, Mepha, Nikitas Gkikopoulos: None declared, Nicolas Perez Gonzalez: None declared, Mike O. Becker Speakers bureau: Mepha, MSD, Novartis, GSK, Bayer and Vifor, Oliver Distler Speakers bureau: Bayer, Boehringer Ingelheim, Medscape, Novartis, Roche, Pfizer, Roche, Sanofi, Consultant of: Abbvie, Acceleron, Alcimed, Amgen, AnaMar, Arxx, AstraZeneca, Baecon, Blade, Bayer, Boehringer Ingelheim, ChemomAb, Corbus, CSL Behring, Galapagos, Glenmark, GSK, Horizon, Inventiva, iQvia, Kymera, Lupin, Medac, Medscape, Miltenyi Biotec, Mitsubishi Tanabe, MSD, Prometheus Biosences, Roche, Roivant, Topadur and UBC, Lilly, Pfizer, Grant/research support from: Kymera, Mitsubishi Tanabe, Michael Krauthammer Speakers bureau: Oncobit, Britta Maurer Speakers bureau: Boehringer-Ingelheim, Medtalk, Pfizer, Roche, Actelion, Mepha, and MSD, Consultant of: Novartis, Boehringer Ingelheim, Janssen-Cilag, Grant/research support from: AbbVie, Protagen, Novartis Biomedical Research

Background and objectives The University of California Los Angeles Scleroderma Clinical Trial Consortium Gastrointestinal Tract Instrument 2.0 (UCLA GIT 2.0) is validated to capture gastrointestinal (GI) tract morbidity in patients with systemic sclerosis (SSc). The aims of this study were to determine in a large SSc cohort if the UCLA GIT 2.0 is able to discriminate patients for whom a rheumatologist with experience in SSc would recommend an esophago-gastro-duodenoscopy (EGD), and if it could identify patients with endoscopically proven esophagitis or with any pathologic finding on EGD. Methods We selected patients fulfilling the ACR/EULAR 2013 criteria for SSc from our EUSTAR center having completed at least once the UCLA GIT 2.0 questionnaire, and we collected data on gastrointestinal symptoms and EGD from their medical charts. We analyzed by general linear mixed effect models several parameters, including UCLA GIT 2.0, considered as potentially associated with the indication of EGD, as well as with endoscopic esophagitis and any pathologic finding on EGD. Results We identified 346 patients (82.7% female, median age 63 years, median disease duration 10 years, 23% diffuse cutaneous SSc) satisfying the inclusion criteria, who completed UCLA GIT 2.0 questionnaires at 940 visits. EGD was recommended at 169 visits. In multivariable analysis, UCLA GIT 2.0 and some of its subscales (reflux, distention/bloating, social functioning) were associated with the indication of EGD. In 177 EGD performed in 145 patients, neither the total ULCA GIT 2.0 score nor any of its subscales were associated with endoscopic esophagitis, nor with any pathologic EGD findings. Conclusions In a real-life setting, the UCLA GIT 2.0 and its reflux subscale were able to discriminate patients with SSc who had an indication for EGD, but did not correlate with findings in EGD. We conclude that, while using the UCLA GIT 2.0 in the routine care of patients with SSc may help the rheumatologist to better understand the burden of GI symptoms in the individual patient, it should not be used as a stand-alone instrument to identify an indication of EGD.

OBJECTIVES To evaluate the feasibility, validity, reliability, and responsiveness of the Hospital Anxiety and Depression Scale (HADS) and to analyse its model structure in patients with systemic sclerosis (SSc). METHODS In this study, 316 SSc patients were included; of these, 159 participated in the responsiveness analysis. Psychometric properties were tested in analogy to the Outcome Measures in Rheumatology (OMERACT) filter and an exploratory and confirmatory factor analysis was performed to examine the structure of HADS. RESULTS The HADS showed adequate feasibility, validity, reliability, and responsiveness to clinically relevant worsening of the disease. For our population of SSc patients, the HADS model with two sub-scales, HADS-A and HADS-D, and a general scale HADS-S, measuring anxiety, depression, and distress, respectively, was most appropriate. The rates of anxiety, depression, mixed anxiety-depressive disorder (MADD) and distress identified by HADS were 32.2%, 25.9%, 18.5%, and 49.5%, respectively, in our cohort. CONCLUSIONS The psychometric properties of the HADS make it useful for screening in SSc, where anxiety, depression, MADD, and distress represent a significant burden to patients.

Additional file 1.