scholarly journals POS1307 ULTRASOUND-DETECTED TENOSYNOVITIS IN ANKLES WITH CLINICALLY ACTIVE DISEASE OF CHILDREN WITH NEW-ONSET JUVENILE IDIOPATHIC ARTHRITIS DOES NOT AFFECT THE CHANCE TO ACHIEVE DISEASE REMISSION

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 935.2-936
Author(s):  
S. Lanni ◽  
O. De Lucia ◽  
S. Orsi ◽  
S. Costi ◽  
G. Beretta ◽  
...  

Background:The ankle is one of the most commonly affected sites in juvenile idiopathic arthritis (JIA). This region has a complex anatomical structure owing to the presence of multiple joint recesses and surrounding tendons. While the prognostic value of ultrasound (US)-detected arthritis has been investigated in recent studies, the role of tenosynovitis in JIA remains still unexplored.Objectives:To investigate: 1) US features of ankle involvement in JIA at disease onset; 2) the predictive value of US-detected tenosynovitis in ankles with clinically active disease of children with new-onset JIA.Methods:The clinical charts of all consecutive patients with new-onset JIA between May 2018 and January 2020 at study centres (Policlinico and G.Pini Hospitals of Milan) and with clinically active ankle disease among the joints affected were reviewed retrospectively. Data on ankle US assessment were retrieved and patients were then stratified as follows: 1) patients with detection on US of isolated arthritis in at least one of the joint recesses of the ankle region; 2) patients with detection on US of tenosynovitis in at least one of the tendon compartments of the ankle irrespective of the presence of concomitant arthritis. For each of these two categories, estimation of patients who were able to achieve clinical disease remission at 12 months since disease onset was evaluated.Results:Twenty-seven new-onset JIA patients were found to have clinical involvement of the ankle among the joints affected. Nine of them (33.3%) showed on US isolated arthritis of the ankle, whereas US-detected tenosynovitis was found in 18 (66.7%) patients. The amount of patients who were able to achieve disease remission at 12-months was the same (66.7%) for both patients with and without US-detected tenosynovitis in the ankle (12/18 and 6/9 patients, respectively). In patients with US-detected tenosynovitis and clinical disease remission at 12 months, the lateral tendon compartment (LTC) was the tendon site more frequently affected by pathology (75.0%). Patients with US-detected tenosynovitis that did not achieve clinical disease remission at follow-up had the highest frequency of tendon pathology on US in the medial tendon compartment (MTC) (83.3%). The anterior tendon compartment was the less frequently affected tendon compartment of the ankle in all patients (33.3% in both patients with and without clinical remission of disease at the 12-months follow-up visit).Conclusion:US-detected tenosynovitis of the ankle is a common finding in patients with new-onset JIA with clinically ankle disease activity and is more frequent than the detection on US of isolated arthritis. The MTC and LTC are the tendon compartments more commonly affected on US. The detection on US of tenosynovitis at disease onset in ankles with clinical disease activity did not seem to affect the change to achieve the overall clinical disease remission compared to patients without tendon pathology but with joint disease in the ankle region.Disclosure of Interests:None declared

2021 ◽  
Author(s):  
Céline La ◽  
Phu Quoc Lê ◽  
Alina Ferster ◽  
Laurence Goffin ◽  
Delphine Spruyt ◽  
...  

Abstract IntroductionIn the management of juvenile idiopathic arthritis (JIA), there is a lack of diagnostic and prognostic biomarkers. This study assesses the use of serum calprotectin (sCal) as a marker to monitor disease activity, and as a classification and prognosis tool of response to treatment or risk of flares in patients with JIA. MethodsEighty-one patients with JIA from the CAP48 multicentric cohort were included in this study, as well as 11 non-pediatric healthy controls. An enzyme-linked immunosorbent assay (ELISA) method was used to quantify sCal with a commercial kit.ResultsPatients with an active disease compared to healthy controls and to patients with inactive disease showed an 8-fold and a 2-fold increased level of sCal respectively. sCal was found to be correlated with the CRP and even more strongly with the ESR. Evolution of DAS28 scores correlated well with evolution of sCal, as opposed to evolution of CRP. With regard to CRP, sCal could differentiate forms with active oligoarthritis from polyarthritis and systemic forms. However, sCal brought an added value compared to the CRP as a prognosis marker. Indeed, patients with active disease and reaching minimal disease activity (according to JADAS) at 6 months following the test had higher sCal levels, while patients with inactive disease had higher sCal levels if a flare was observed up to 3 to 9 months following the test.ConclusionsThis study confirms the potential uses of serum calprotectin as a biomarker in the diagnosis and follow-up of JIA.


2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 1396.2-1396
Author(s):  
N. Ben Chekaya ◽  
S. Bouden ◽  
A. Ben Tekaya ◽  
O. Ben Saidane ◽  
R. Tekaya ◽  
...  

Background:Juvenile idiopathic arthritis (JIA) is the most common chronic rheumatic disease of unknown aetiology in childhood. Approximately 40- 60% of patients with juvenile idiopathic arthritis (JIA) have continuous or recurrent disease activity extending into adulthood.Objectives:The aim of this study was to evaluate the clinical, biological and radiological course of JIA in adulthood.Methods:A retrospective study including 35 patients was conducted between 2010 and 2019. The patients enrolled met the ILAR criteria for the diagnosis of JIA. Patients with JIA older than 20 years were included. Data regarding sociodemographic features, Physical growth, disease activity, biological and radiological parameters were analysed.Results:Thirty one patients were recruited. The sex ratio was 0.53. The mean age at the time of the study was 38.9 years [20-69]. The mean age of the disease onset was 9 years [3-16]. These patients were assigned to discrete JIA categories: rheumatoid factor positive polyarthritis (47.7%), rheumatoid factor negative polyarthritis (23.9%), oligoarthritis (14.2%), enthesitis-related arthritis (9.5%), and psoriatic arthritis (4.7%).A failure to thrive was seen in 33.3% of patients. Overweight and obesity were found in 38% and 19% of patients, respectively. Biological inflammatory syndrome was noted in 52% of patients, and 65.2% had active disease. Hip involvement was noted in 43.5% of patients and 17.4% of them had a total hip replacement. Sixteen patients had neck pain and the imagining showed an atloid-axoid dislocation in 50% of them. Radiographs showed a joint destruction in 60.9% of patients and a wrist arthritis was the most frequent involvement.Conclusion:Most of our included patients maintain active disease and have functional impairment in adulthood.Disclosure of Interests:None declared


2021 ◽  
Vol 19 (1) ◽  
Author(s):  
Ellen Dalen Arnstad ◽  
◽  
Mia Glerup ◽  
Veronika Rypdal ◽  
Suvi Peltoniemi ◽  
...  

Abstract Background To study fatigue in young adults with juvenile idiopathic arthritis (JIA) 18 years after disease onset, and to compare with controls. Methods Consecutive children with onset of JIA between 1997 and 2000, from geographically defined areas of Norway, Sweden, Denmark and Finland were followed for 18 years in a close to population-based prospective cohort study. Clinical features, demographic and patient-reported data were collected. Inclusion criteria in the present study were a baseline visit 6 months after disease onset, followed by an 18-year follow-up with available self-reported fatigue score (Fatigue Severity Scale (FSS), 1–7). Severe fatigue was defined as FSS ≥4. For comparison, Norwegian age and sex matched controls were used. Results Among 377 young adults with JIA, 26% reported severe fatigue, compared to 12% among controls. We found higher burden of fatigue among participants with sleep problems, pain, poor health, reduced participation in school/work, physical disability, active disease, or use of disease-modifying anti-rheumatic drugs (DMARDs)/biologics/systemic steroids. In contrast, participants without these challenges, had fatigue scores similar to controls. Active disease assessed at all three time points (baseline, 8-year and 18-year follow-up) was associated with higher mean fatigue score and higher percentage of severe fatigue compared to disease courses characterized by periods of inactive disease. Predictors of fatigue at the 18-year follow-up were female sex and diagnostic delay of ≥6 months at baseline, and also pain, self-reported poor health, active disease, and previous/ongoing use of DMARDs/biologics at 8 years. Conclusions Fatigue is a prominent symptom in young adults with JIA, with higher fatigue burden among participants with poor sleep, pain, self-reported health problems, active disease, or use of DMARDs/biologics. Participants without these challenges have results similar to controls. Patient- and physician-reported variables at baseline and during disease course predicted fatigue at 18-year follow-up.


2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 504.1-504
Author(s):  
V. Kachkovska ◽  
A. Kovchun ◽  
E. Prystupa ◽  
L. Prystupa

Background:Nailfold capillaroscopy (NFC) is a useful, noninvasive, widely available diagnostic tool in rheumatology practice. We commonly use it to describe patterns of abnormalities in systemic sclerosis however we have enough data which proves NFC usefulness for monitoring idiopathic inflammatory myopathies (IIM) considering it as diagnostic tool, monitoring of disease activity and treatment efficiency. Despite evident clinical relevance of NFC in IIM patients we still do not have clear capillaroscopic images for IIM definition.Objectives:That’s why, the goal of our research was aimed to analyze capillaroscopic peculiarities among IIM patients and find possible associations with clinical and activity data.Methods:69 patients with IIM were examined and 47 IIM patients with capillaroscopic alterations were included in the study, 26 patients with dermatomyositis (DM) and 21 patients with polymyositis (PM) according to the Targoff Criteria (1997). NFC we performed usingDino-Lite CapillaryScope with 200 magnification. We assessed nailfold capillary density (NCD), presence of microhemorrhages, giant, dilated and ramified capillaries, scleroderma patterns (defined as an early, active or late pattern) and neovascular pattern (defined as an active and late scleroderma patterns). To asses disease activity we use Manual Muscle Testing 8 (MMT8), Health Assessment Questionnaire (HAQ), Myositis Disease Activity Assessment Tool (MDAAT), Cutaneous Dermatomyositis Disease Area and Severity Index (CDASI), physician’s VAS, patient’s VAS, serum muscle enzymes levels. We divided patients into 4 groups: 1stgroup – 17 DM patients with active disease (8 of them with newly onset disease), 2ndgroup included 9 DM patients with inactive disease, 3rdgroup – 11 PM patients with active disease (5 of them with newly onset disease) and 4thgroup included 10 PM patients with inactive disease.Results:The most common finding was low NCD, 70% of all patients had NCD lower than 6 per 1 mm. NCD for the 1stgroup was 4,4±1,12, 2ndgroup – 6,0±1,0, 3rdgroup – 5,8±1,1, 4thgroup – 9,2±2,1 (F=26,27, p<0,001). Hemorrhages were significantly more common among DM patients and active disease and were observed among 47,1% (p=0,036,χ2=8,574) with no significant difference with regard to the disease onset. Analyzing scleroderma patterns, among 1stgroup of patients – 17,6% had early, 47,1% – active, 35,3% – late pattern, in the 2ndgroup – 66,7% had early pattern, in the 3rdgroup – 27,3% patients with early and 18,2% with active pattern and in the 4thgroup – 50% of patients presented with early pattern (p=0,001,χ2=31,87). Neovascular pattern was found significantly more often among patients with active DM (p=0,001) with no regard to the disease onset. No statistically significant difference in giant and ramified capillaries distribution was found.Conclusion:According to our results, we can admit that the most common capillaroscopic finding was decreased NCD, which were significantly lower among patients with active DM, the same as microhemorrhages and neovascular scleroderma pattern. This data suggests that NCD, microhemorrhages and neovascular scleroderma pattern could be consider as biomarkers of DM activity but not PM, therefore more detailed research with larger numbers of patients are required.Disclosure of Interests:None declared


2019 ◽  
Vol 17 (1) ◽  
Author(s):  
Mia Glerup ◽  
◽  
Steffen Thiel ◽  
Veronika Rypdal ◽  
Ellen Dalen Arnstad ◽  
...  

Abstract Background To determine the serum levels of the lectin pathway proteins early in the disease course and 17 years after disease onset and to correlate the protein levels to markers of disease activity in participants from a population-based Nordic juvenile idiopathic arthritis (JIA) cohort. Additionally, to assess the predictive value of lectin pathway proteins with respect to remission status. Methods A population-based cohort study of consecutive cases of JIA with a disease onset from 1997 to 2000 from defined geographical areas of Finland, Sweden, Norway and Denmark with 17 years of follow-up was performed. Clinical characteristics were registered and H-ficolin, M-ficolin, MASP-1, MASP-3, MBL and CL-K1 levels in serum were analyzed. Results In total, 293 patients with JIA were included (mean age 23.7 ± 4.4 years; mean follow-up 17.2 ± 1.7 years). Concentrations of the lectin protein levels in serum were higher at baseline compared to the levels 17 years after disease onset (p ≤ 0.006, n = 164). At baseline, the highest level of M-ficolin was observed in systemic JIA. Further, high M-ficolin levels at baseline and at 17-year follow-up were correlated to high levels of ESR. In contrast, high MASP-1 and MASP-3 tended to correlate to low ESR. CL-K1 showed a negative correlation to JADAS71 at baseline. None of the protein levels had prognostic abilities for remission status 17 years after disease onset. Conclusion We hypothesize that increased serum M-ficolin levels are associated with higher disease activity in JIA and further, the results indicate that MASP-1, MASP-3 and CL-K1 are markers of inflammation.


2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 1399.3-1400
Author(s):  
H. Bettaieb ◽  
H. Ferjani ◽  
K. Maatallah ◽  
H. Boussaa ◽  
D. Kaffel ◽  
...  

Background:Atlanto-axial instability (AAI) is a serious complication during Juvenile Idiopathic Arthritis (JIA). It can lead to severe neurological morbidity or mortality if left untreated (1).Objectives:To determine the prevalence of AAI in patients with JIA and to identify factors associated with an increased risk of its occurrence.Methods:A retrospective monocentric study was carried out on JIA patients (ILAR criteria). Data, including age at disease onset, JIA type, disease activity at AAI diagnosis and treatment were collected. Disease activity at JIA diagnosis was evaluated by JADAS10 (Juvenile Arthritis Disease Activity Score) in poly and oligoarticular subtypes and by BASDAI (Bath Ankylosing Spondylitis Disease Activity Index) in arthritis related enthesitis form. Standard radiographs of the cervical spine were analyzed. The data were analyzed using the SPSS statistical package. A p value < 0.05 was considered significant.Results:We enrolled 48 JIA (31 male and 17 female) with a mean age at disease onset of 11.2 ± 3.8 years. The median disease duration was 84 months [2-408]. The median JIA diagnosis delay was 8 months [1-108]. The JIA subgroups were in decreasing order of frequency: Enthesitis-related Arthritis (n=32), Polyarticular RF- (n=4), Polyarticular RF+ (n=2), Oligoarticular (n=6), Systemic (n=2), Psoriatic Arthritis (n=1) and Undifferentiated (n=1).At diagnosis, median ESR and CRP were 44 mm/hour [2-100] and 24 mg/l [2-86] respectively. Median JADAS10 score was 4 [0-21]. Median BASDAI score was 6.2 [2-9.4].At follow-up, five patients (10.4%) had atlantoaxial subluxation and 17 had coxitis (43.8%).At bone densitometry, 45% of patients had osteroposis and 27.5% had osteopenia.An agreement was assessed between a long diagnosis delay and the following parameters: male gender (p=0.04) and osteoporosis (p=0.018). A Significant positive correlation was found between delay in JIA diagnosis and BASDAI score (p=0.047, r=0.63). No association was found between JIA diagnosis delay and JADAS score (p=0.56). Neither ESR (p=0.19) nor CRP (p=0.42) was associated with JIA diagnosis delay.Finally, no link was observed with the occurrence of hip arthritis (p=0.281) or atlantoaxial subluxation (p=0.137).Conclusion:In our study, the prevalence of AAI was 10.4%. Prolonged corticosteroid use and elevated inflammatory markers were the major factors associated with an increased risk of upper cervical spine involvement. Hence, targeted treatments are required to prevent cervical spine instability.References:[1]Hospach T, Maier J, Müller-Abt P, Patel A, Horneff G, von Kalle T. Cervical spine involvement in patients with juvenile idiopathic arthritis - MRI follow-up study. Pediatr Rheumatol Online J. 2014;12:9.Disclosure of Interests:None declared


2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1207.2-1207
Author(s):  
A. García Fernández ◽  
A. Briones-Figueroa ◽  
L. Calvo Sanz ◽  
Á. Andreu-Suárez ◽  
J. Bachiller-Corral ◽  
...  

Background:Biological therapy (BT) has changed the treatment and perspectives of JIA patients but little is known about when is the best moment to start BT and the impact of this prompt iniciation.Objectives:To analyze the response to BT of Juvenile Idiophatic Arthritis (JIA) patients according to the time when the BT was started.Methods:A retrospective, descriptive study was conducted on JIA patients followed up in a referal hospital that started BT up to 24 months after diagnosis from 2000 to 2018. Disease activity was measured, at 2 years after diagnosis, according to Wallace criteria for remission (absence of: active arthritis, active uveitis, fever, rash or any other manifestation attributable to JIA, normal CRP and ESR, PGA indicating no active disease) for at least 6 months.Results:55 JIA patients that started BT up to 24 months from diagnosis were analyzed. 69,1% were girls with a median age at diagnosis of 8 years old IQR(3-13), median age at the start of BT of 9 years old IQR(3-13). Regarding JIA categories: 25,5% were Oligoarticular Persistent (OligP), 18,2% Systemic JIA (sJIA), 16,4% Entesitis related Arthritis (ERA), 12,7% Psoriatic Arthritis (APso) and Polyarticular RF- (PolyRF-), 5,5% Oligoarticular Extended (OligE) and Polyarticular RF+ (PolyRF+), 3,6% Undifferentiated (Und). 20% of patients had uveitis during followup. Conventional DMARD (cDMARD) was indicated in 83,6% of patients (95,7% Methotrexate) at diagnosis [median 0 months IQR(0-2,3)]. At the end of followup (2 years) only 30,9% of patients continued with cDMARDs. The main causes of discontinuation were: adverse events (46,7%), remission (36,7%). TNF inhibitors were precribed in 81,8% of patients and 18,2% of patients recieved two BT during the first 2 years from diagnosis. 54,5% of BT were indicated during the first 6 months from diagnosis, 27,3% from 7 to 12 months, 12,7% from 13 to 18 months, 5,5% from 19 to 24 months.After 2 years from diagnosis, 78,2% of patients were on remission and 21,8% active. Among patients with active disease: 75% had arthritis, 16,7% had uveitis and 8,3% had both. There were no differences regarding disease activity among patients with uveitis and neither taking cDMARDs. Regarding JIA categories: 66,7% of OligE, 57,1% of PolyRF- and 57,1% of APso patients were active at 2 years from diagnosis when compared to the other categories (p=0.004).Patients on remission at 24 months from diagnosis started sooner the BT than active patients [CI 95% (0,46-8,29) p=0,029]. The time when the BT was started was correlated to the activity at 2 years (K= 0,294 p=0,029). When the BT was prescribed after 7,5months from diagnosis it was correlated, in a COR curve, with a higher probability of active disease at 2 years (S= 0,67 E= 0,63). There was a correlation, among patients on remission at 2 years, between prompt start of BT and less time to reach remission (K= -0,345 p=0,024). Patients with active disease at 2 years, regardless of moment of BT iniciation, required more BT during follow-up (p=0,002).Conclusion:Prompt iniciation of BT was correlated with a better outcome. JIA patients that started BT early after diagnosis had a higher probability of remission after 2 years. Starting BT after 7,5 months was correlated with a higher probability of active disease at 2 years. Active disease at 24 months was correlated with persistent active disease during follow-up.Disclosure of Interests:None declared


2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 492.2-492
Author(s):  
K. Mandai ◽  
M. Tada ◽  
Y. Yamada ◽  
T. Koike ◽  
T. Okano ◽  
...  

Background:Rheumatoid arthritis (RA) patients have a high frequency of sarcopenia, and they commonly have reduced physical function. We previously reported that the prevalence of sarcopenia was 28%, that of frailty was 18.9%, and that of pre-frailty was 38.9% in RA patients1,2, and 13.2% of RA patients developed sarcopenia within a year 3.Objectives:To investigate the risk factors for new onset of sarcopenia, locomotive syndrome, and frailty in patients with RA and the course of each disease.Methods:Two-year follow-up data from the rural group of the prospective, observational CHIKARA study were used. Sarcopenia was diagnosed using the criteria of the Asian Working Group for Sarcopenia 2014, locomotive syndrome was diagnosed using locomotive 5, and frailty was diagnosed using the basic checklist. New onset of the disease over the 2-year follow-up period was studied, excluding cases that had the disease at baseline. Improvement was defined as cases with disease at baseline that no longer met the diagnostic criteria after 2 years. Differences in the characteristics of each disease were tested using the Chi-squared test and the paired t-test.Results:The 81 patients with RA (82.7% female) had mean age 66.9±11.5 years, mean DAS28-ESR 2.9±1.2, methotrexate use in 81.5% (with a dose of 9.9±2.7 mg/week), and glucocorticoid (GC) use in 22.2% (with a dose of 3.1±1.7 mg/week). The baseline prevalence was 44.4% for sarcopenia, 35.8% for locomotive syndrome, and 25.9% for frailty, and the new onset rate was 4.4% for sarcopenia, 15.4% for locomotive syndrome, and 13.3% for frailty. Of the patients with each disease at baseline, 36.1% had sarcopenia, 20.7% had locomotive syndrome, and 33.3% had frailty, and of those with each disease at 2 years, 36.1% had sarcopenia, 20.7% had locomotive syndrome, and 33.3% had frailty. The new onset sarcopenia and locomotive syndrome groups had significantly higher rates of GC use (p=0.036, p=0.007, paired t-test) and significantly higher doses (p=0.01, p=0.001, paired t-test) than the groups without new onset sarcopenia and locomotive syndrome. High baseline disease activity was an independent predictor of new onset of locomotive syndrome on multivariate logistic regression analysis (OR=3.21, p=0.015).Conclusion:The new onset rates at 2 years were 4.4% for sarcopenia, 15.4% for locomotive syndrome, and 13.3% for frailty. In the new onset sarcopenia and locomotive syndrome groups, both GC use and dosage were significantly higher.References:[1]Tada M, et al. Matrix metalloprotease 3 is associated with sarcopenia in rheumatoid arthritis - results from the CHIKARA study. Int J Rheum Dis. 2018 Nov;21(11):1962-1969.[2]Tada M, et al. Correlation between frailty and disease activity in patients with rheumatoid arthritis: Data from the CHIKARA study. Geriatr Gerontol Int. 2019 Dec;19(12):1220-1225.[3]Yamada Y, et al. Glucocorticoid use is an independent risk factor for developing sarcopenia in patients with rheumatoid arthritis: from the CHIKARA study. Clin Rheumatol. 2020 Jun;39(6):1757-1764.Disclosure of Interests:None declared


2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 409.1-409
Author(s):  
T. Minier ◽  
V. Lóránd ◽  
Z. Bálint ◽  
D. Komjati ◽  
G. Nagy ◽  
...  

Background:Disease activity assessment is crucial in defining the appropriate therapy and to monitor the efficacy of treatment in systemic sclerosis.Objectives:We aimed to test the performance of the ’old’ European Scleroderma Trials and Research Group (EUSTAR) Activity Index (old-AI) (1), the ’new’ EUSTAR activity index (new-AI) (2), and the scleroderma activity index derived from the old-AI (Pecs-AI) (3). We compared the three indices to the disease activity based on the physician’s global assessment (PGA). We also assessed the correlations with the change in modified Rodnan Skin Score (MRSS), FVC and arthritis after one year follow-up.Methods:We evaluated 77 patients (50 diffuse /dcSSc/ and 27 limited cutaneous SSc /lcSSc/ patients) from a single tertiary clinical center. Cohort enrichment was performed to increase the number of patients with early disease and dcSSc. Seventy-two patients were re-evaluated after one year. Nine patients had overlap syndromes: rheumatoid arthritis (n=3), Sjögren syndrome (n=2), polymyositis (n=2), and mixed connective tissue disease (n=2). The overall disease activity was evaluated using both composite indices (old-AI, Pecs-AI, new-AI) and the PGA of disease activity, based on the blinded evaluation of a single physician (LV). In addition to the minimal essential data from the EUSTAR database we also performed detailed assessment of the musculoskeletal involvement evaluating measures of hand function, DAS28 scores, and the Clinical Disease Activity Index (CDAI) (4).Results:Three times more patients with active disease were identified by the new-AI compared to the old-AI at baseline investigation (n=37, 48.7%, vs. n=11, 14.3%). Two patients (18%) with active disease based on the old-AI were missed by the new-AI. Pecs-AI index identified 15 patients (19.5%) with active disease (cut-off >2.75 points). Active disease was equally frequent in dcSSc and lcSSc patients based on old-AI, but was more frequent in dcSSc patients based on the new-AI in the whole cohort, and also after excluding overlap cases.Patients with active disease based on the old-AI had more frequently rheumatoid factor (6/9, vs. 12/45, p=0.047), and DLCO<70% (11/11, vs. 36/65, p<0.01). Active disease based on the new-Al was associated with current cyclophosphamide treatment (9/37, vs.2/39, p=0.023), and diabetes mellitus (7/30, vs. 0/39, p<0.01). The PGA correlated moderately at both baseline and one year follow-up examination with the old-AI (rho: 0.519, and rho: 0.692, respectively, p<0.001), the new-AI (rho: 0.401, and rho: 0.429, respectively, p<0.001), and the Pecs-AI (rho: 0.425, and rho: 0.593, respectively, p<0.001).CDAI correlated significantly with the old-AI (rho: 0.345, and rho: 0.283, respectively, p<0.05) and the Pecs-AI (rho: 0.363, and rho: 0.324, respectively, p<0.05) at both the baseline and one-year follow-up investigations, but showed no consistent correlation to the new-AI or PGA.Conclusion:The two validated disease activity indices indentify different patient groups. Joint involvement is potentially underrepresented in the new EUSTAR activity index. Active disease is also present in lcSSc and should be assessed regularly in these patients.References:[1]Valentini G, et al. Ann Rheum Dis 2003; 62: 901-3.[2]Valentini G, et al. Ann Rheum Dis 2017;76:270–276.[3]Minier T, et al. Rheumatology (Oxford) 2010;49(6):1133-45.[4]Lorand V, et al. Rheumatology (Oxford). 2016;55(10):1849-58.Acknowledgments:This work was supported by the EU Seventh Framework Program [FP7/2007-2013] under Grant Agreement n° 305495 (DeSScipher), by the Hungarian Scientific Research Fund (contract n°: 112939), and the EU under the Grant Agreement n° PEPSYS GINOP-232-15-2016-00050.Disclosure of Interests:Tünde Minier Speakers bureau: Actelion, Abbvie, MSD, Pfizer, Lilly, Roche, Veronika Lóránd: None declared, Zsófia Bálint: None declared, Dalma Komjati: None declared, Gabriella Nagy Speakers bureau: MSD, Antonietta Kovács: None declared, Orsolya Koncz: None declared, Cecília Varjú Consultant of: Boehringer Ingelheim RCV GmbH & Co KG, Speakers bureau: Lilly, László Czirják Consultant of: Actelion, BI, Roche-Genentech, Lilly, Medac, Novartis, Pfizer, Bayer AG, Gabor Kumanovics Consultant of: Boehringer, Teva, Speakers bureau: Roche, Lilly, Novartis, Balazs Nemeth: None declared


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