Clinical Disease Activity Score Research Articles

Disease Damage in Juvenile Idiopathic Arthritis.

To estimate the prevalence and predictors of disease-related damage in children with juvenile idiopathic arthritis (JIA) in a resource-limited setting. A single-centre study was conducted from January 2021 - December 2022. Children (≤ 18 y) diagnosed with JIA as per International League of Associations for Rheumatology (ILAR) criteria, with a disease duration of more than one year, were enrolled for this study. The articular and extra-articular damage was assessed using the juvenile arthritis damage index (JADI) and modified JADI scores. Disease activity and disabilities were evaluated using the clinical juvenile arthritis disease activity score (cJADAS) and Childhood Health Assessment Questionnaire (CHAQ). One hundred and five children [44% (n = 42) boys] with JIA were enrolled in the study. The mean (SD) age of children at enrolment was 158 (46.2) mo. The median (IQR) disease duration was 48 (36-72) mo. Articular damage (JADI score ≥ 1) was present in 48.6%, and extra-articular damage (JADI-E ≥ 1) was observed in 21.9% of children. Half of the children (n = 22) with enthesitis-related arthritis (ERA) had joint damage (modified JADI score ≥ 1). Four children had ocular damage due to uveitis. Among the factors associated with articular damage, the odds of articular damage were high in those with positive rheumatoid factor (RF) and/ or anti-cyclic citrullinated peptides (CCPs) [OR: 4.4, 95% CI (1.00-19.60)]. 48.6% of children with JIA had articular damage, while 21.9% of the children had extra-articular damage. Children with RF and/ or anti-CCP positivity are associated with higher odds of joint damage.

Subjective and objective sleep quality assessment in adolescent patients with inflammatory bowel disease

ObjectivesThere is a link between inflammatory bowel disease (IBD) and poor sleep quality that is presumed to be multifactorial. The purpose of this study was to identify factors that impact sleep quality in this group including clinical disease activity, depression, anxiety, quality of life, and disordered social media use through questionnaires. MethodsThis prospective study analyzed sleep quality in adolescent patients ages 13 to 18 with a diagnosis of IBD using objective data from wrist actigraphy and subjective report from the Pittsburgh Sleep Quality Index (PSQI). Additional screeners including the Short Pediatric Crohn's Disease Activity Index or Pediatric Ulcerative Colitis Activity Index, Pediatric Quality of Life Inventory Gastrointestinal Symptoms Module, Generalized Anxiety Disorder 7-item screener, Patient Health Questionnaire depression screener, and Social Media Disorder scale were also collected. ResultsTwenty-three subjects enrolled and 16 completed questionnaires. 62.5 % of participants were in clinical remission and the remaining 37.5 % had mild to severe clinical IBD activity. Poor subjective sleep quality was associated with a shorter duration of sleep time and an increased clinical disease activity score. Patients in clinical remission slept for a longer duration than those with mild IBD activity. ConclusionsThis study did not identify a significant relationship between sleep quality and psychosocial factors in the adolescent population with IBD, though their influence cannot be discounted. There was a connection between the presence of disease activity and shorter sleep duration. Similarly, PSQI scores correlated with sleep time but not sleep quality.

Assessing disparities through missing race and ethnicity data: results from a juvenile arthritis registry.

Ensuring high-quality race and ethnicity data within the electronic health record (EHR) and across linked systems, such as patient registries, is necessary to achieving the goal of inclusion of racial and ethnic minorities in scientific research and detecting disparities associated with race and ethnicity. The project goal was to improve race and ethnicity data completion within the Pediatric Rheumatology Care Outcomes Improvement Network and assess impact of improved data completion on conclusions drawn from the registry. This is a mixed-methods quality improvement study that consisted of five parts, as follows: (1) Identifying baseline missing race and ethnicity data, (2) Surveying current collection and entry, (3) Completing data through audit and feedback cycles, (4) Assessing the impact on outcome measures, and (5) Conducting participant interviews and thematic analysis. Across six participating centers, 29% of the patients were missing data on race and 31% were missing data on ethnicity. Of patients missing data, most patients were missing both race and ethnicity. Rates of missingness varied by data entry method (electronic vs. manual). Recovered data had a higher percentage of patients with Other race or Hispanic/Latino ethnicity compared with patients with non-missing race and ethnicity data at baseline. Black patients had a significantly higher odds ratio of having a clinical juvenile arthritis disease activity score (cJADAS10) of ≥5 at first follow-up compared with White patients. There was no significant change in odds ratio of cJADAS10 ≥5 for race and ethnicity after data completion. Patients missing race and ethnicity were more likely to be missing cJADAS values, which may affect the ability to detect changes in odds ratio of cJADAS ≥5 after completion. About one-third of the patients in a pediatric rheumatology registry were missing race and ethnicity data. After three audit and feedback cycles, centers decreased missing data by 94%, primarily via data recovery from the EHR. In this sample, completion of missing data did not change the findings related to differential outcomes by race. Recovered data were not uniformly distributed compared with those with non-missing race and ethnicity data at baseline, suggesting that differences in outcomes after completing race and ethnicity data may be seen with larger sample sizes.

Characterizing the human intestinal chondroitin sulfate glycosaminoglycan sulfation signature in inflammatory bowel disease

The intestinal extracellular matrix (ECM) helps maintain appropriate tissue barrier function and regulate host-microbial interactions. Chondroitin sulfate- and dermatan sulfate-glycosaminoglycans (CS/DS-GAGs) are integral components of the intestinal ECM, and alterations in CS/DS-GAGs have been shown to significantly influence biological functions. Although pathologic ECM remodeling is implicated in inflammatory bowel disease (IBD), it is unknown whether changes in the intestinal CS/DS-GAG composition are also linked to IBD in humans. Our aim was to characterize changes in the intestinal ECM CS/DS-GAG composition in intestinal biopsy samples from patients with IBD using mass spectrometry. We characterized intestinal CS/DS-GAGs in 69 pediatric and young adult patients (n = 13 control, n = 32 active IBD, n = 24 IBD in remission) and 6 adult patients. Here, we report that patients with active IBD exhibit a significant decrease in the relative abundance of CS/DS isomers associated with matrix stability (CS-A and DS) compared to controls, while isomers implicated in matrix instability and inflammation (CS-C and CS-E) were significantly increased. This imbalance of intestinal CS/DS isomers was restored among patients in clinical remission. Moreover, the abundance of pro-stabilizing CS/DS isomers negatively correlated with clinical disease activity scores, whereas both pro-inflammatory CS-C and CS-E content positively correlated with disease activity scores. Thus, pediatric patients with active IBD exhibited increased pro-inflammatory and decreased pro-stabilizing CS/DS isomer composition, and future studies are needed to determine whether changes in the CS/DS-GAG composition play a pathogenic role in IBD.

Long-term safety and efficacy of upadacitinib versus adalimumab in patients with rheumatoid arthritis: 5-year data from the phase 3, randomised SELECT-COMPARE study

ObjectivesTo assess the safety and efficacy of upadacitinib versus adalimumab from SELECT-COMPARE over 5 years.MethodsPatients with rheumatoid arthritis and inadequate response to methotrexate were randomised to receive upadacitinib 15 mg...

Chronic non-bacterial osteomyelitis (CNO) and chronic recurrent multifocal osteomyelitis (CRMO) with a focus on pamidronate therapy.

Chronic recurrent multifocal osteomyelitis (CRMO), also called chronic nonbacterial osteomyelitis (CNO) or nonbacterial osteomyelitis (NBO), is a rare autoinflammatory bone disease of unknown etiology. However, the number of patients properly diagnosed would increase with better knowledge of the disease. In this regard, whole-body magnetic resonance imaging (WB MRI) has been found to be a better predictor of active lesions than clinical examination. Importantly, the RINBO index (radiologic index for NBO) quantifies the involvement based on the WB MRI. Further, a chronic nonbacterial osteomyelitis MRI scoring (CROMRIS) has been developed as an online tool for assessing WB MRI. The therapy consists of non-steroidal anti-inflammatory drugs (NSAIDs), bisphosphonates (pamidronate, zoledronate, etc.) and other drugs, including biologics. Pamidronate is an appropriate and safe therapy. The first pilot prospective randomised controlled trial (RCT) on pamidronate vs. placebo was carried out in adults. No RCT has been done in children yet. Besides RCTs, there are a number of issues to be explored in future, i.e. predictors of therapy effect, optimal therapy duration, predictors of therapy discontinuation and evaluation of optimal therapy protocol. Recently, the CNO clinical disease activity score (CDAS) was constructed and validated but the classification criteria are still being developed. As collaboration on this rare disease is essential, a prospective Chronic Nonbacterial Osteomyelitis International Registry (CHOIR) was established to generate future comparative effectiveness research data.

Clinical disease activity index versus disease activity score (28 joints) for assessment of disease activity in obese patients of rheumatoid arthritis

Background: Measurement of disease activity by Disease Activity Score 28 joints -Erythrocyte Sedimentation Rate (DAS28-ESR), Disease Activity Score (28 joints)-C-Reactive Protein (DAS28-CRP), and Clinical Disease Activity Index (CDAI) has become an integral part of management of Rheumatoid Arthritis (RA), by ‘Treatment to Target’ approach. With the exception of CDAI, the other two use inflammatory markers ESR and CRP to measure disease activity. Obesity is also known to increase inflammatory markers like CRP. We undertake this study to examine if obesity confounds the disease activity measurement in RA leading to overestimation of disease activity. Methods: A cross-sectional observation study was conducted on one hundred patients of RA (40 obese and 60 non obese) in remission or low disease activity as defined by CDAI. They were divided into obese and non-obese groups based on Indian standards (BMI>25kg/m2). ESR and CRP were measured in both the groups. DAS28-ESR and DAS28-CRP were calculated and compared using relevant statistical tests. Results: DAS28-ESR and DAS-28-CRP scores were significantly higher in the obese subjects, despite both groups having comparable CDAI scores. Similar findings were also observed with inflammatory markers ESR and CRP, both being higher in obese patients. Conclusions: We conclude that indices incorporating inflammatory markers, like DAS28 overestimate disease activity in obese RA patients. Treatment decisions regarding escalation or addition of DMARDs should be taken after considering the same. CDAI appears to be better suited for disease activity measurements in obese RA patients as compared to DAS 28.

OA02 Longitudinal high throughput proteomics identifies novel proteins associated with inflammation and future response in new-onset, treatment-naïve rheumatoid arthritis

Abstract Background/Aims Despite the availability of effective targeted therapies, only 20-30% achieve low disease activity and even fewer attain remission. There remains a pressing need to identify meaningful biomarkers and disease endotypes to enable precision medicine. We aimed to identify serum protein biomarkers that associate with RA inflammation and future remission in a treatment-naïve, new-onset RA trial cohort. Methods Serum from 105 patients from the ‘VEDERA’ trial (first-line tumour necrosis factor inhibitor + methotrexate [TNFi + MTX] versus methotrexate treat-to-target [MTX-TT]) at baseline, week 24 and 48 were analysed for 92 proteins using the Olink Target 96 Inflammation panel. Clinical and MSUS assessments completed at baseline, weeks 24 and 48 were used for the analyses. Clinical remission was defined as DAS28-ESR ≤ 2.6 and joint-level remission as absence of power Doppler ultrasound synovitis (PDUS). After adjusting for age, gender and seropositive status, Bayesian mixed effects regression (posterior estimates and 95% credible intervals reported) was used to identify association of (i) proteins with clinical disease activity (DAS28-ESR) and joint-level inflammation (total power Doppler ultrasound synovitis (PDUS) scores] and (ii) baseline proteins with future (week 24 or 48) clinical and joint-level remission. Results Longitudinally, out of 92 proteins, 10 and 14 were significantly associated with DAS28-ESR and total PDUS scores respectively, with 3 proteins in common: leukaemia inhibitory factor receptor (LIF-R); fractalkine (CX3CL1); interleukin-10 receptor alpha (IL-10RA). Baseline levels of 9 proteins were associated with future clinical remission (week 24 = 6 proteins; week 48 = 3 proteins) while 6 were associated with future PDUS status (week 24 = 4 proteins; week 48 = 3 proteins). Proteins that were associated with inflammation longitudinally and whose baseline levels associated with future remission included IL-17C, fibroblast growth factor 5 (FGF-5), lymphotoxin alpha and TNF. Posterior estimates and 95% credible intervals for above proteins are reported in Table 1. Conclusion Using longitudinal high-throughput proteomics in a treatment-naïve early onset RA cohort, this study sheds new light on known and novel protein associates of inflammation and future remission. With future validation, these biomarkers could provide mechanistic understanding and allow for individualised treatment strategies to improve outcomes. Disclosure R. Shukla: None. R. Wakefield: None. A. Tan: None. P. Emery: Consultancies; BristolMyersSquibb, AbbVie, Gilead, Galapagos, Eli Lilly, MSD, Pfizer, Novartis, Roche and Samsung. Grants/research support; AbbVie, Eli Lilly, Novartis, BristolMyersSquibb, Pfizer and Roche, outside the submitted work. D. Plant: None. M.H. Buch: Consultancies; AbbVie, Arxx Therapeutics, Boehringer Ingelheim, Galapagos, Gilead, and Pfizer; and speaker fees from AbbVie, CESAS Medical, Galapagos, Gilead, Medistream, and Pfizer. Grants/research support; grant support from Gilead.

Doporučení Pracovní skupiny pro idiopatické střevní záněty pro diagnostiku Crohnovy choroby

Background and aims: Recently, the first subcutaneous infliximab (IFX), CTP-13, was approved in the indication of IBD. Pivotal study established noninferiority of subcutaneous infliximab to intravenous formulation in terms of efficacy, with no unexpected safety signals observed. The aims of the study were to evaluate persistency of treatment, pharmacokinetics and safety following a switch from intravenous to subcutaneous infliximab treatment. Methods: This retrospective, single-centre cohort study, recruited all Crohn’s disease (CD) and ulcerative colitis (UC) patients who transitioned from intravenous to subcutaneous IFX between September 2022 and September 2023. Clinical disease activity according to HBI and pMayo, sIBDQ, faecal calprotectin, drug persistence, safety and pharmacokinetics were evaluated at week 24 and 56 after the switch to subcutaneous infliximab. Results: A number of 107 patients was included (63 CD and 44 UC). Quality of life and clinical disease activity scores remained unchanged both in CD and UC. Faecal calprotectin decreased significantly in CD but not in UC. Patients that had been on standard compared to intensified IV infliximab dosing displayed similar outcomes on standard SC dosing. No serious adverse events occurred. Transient local injection reactions were experienced by 4.7% of patients. Median infliximab trough levels significantly increased after the switch to subcutaneous formulation. Conclusion: The switch from intravenous to subcutaneous infliximab is a safe and effective option for the treatment of inflammatory bowel diseases including those with active disease and/ or on intensified intravenous infliximab regime.

P784 Efficacy of ustekinumab in biologically naïve patients with Inflammatory Bowel Disease

Abstract Background In recent years, there are several new treatment options for inflammatory bowel disease (IBD). Tumour necrosis factor α inhibitors have been joined by novel advanced therapies (vedolizumab, ustekinumab (UST) and Janus kinase inhibitors). These drugs are mostly used as second-line treatments after tumor necrosis factor α inhibitors, with subsequent poorer efficacy. There are few data available on the efficacy of first-line treatment with UST. The aim of our study was to assess efficacy of UST in biologically naïve patients with IBD. Methods All patients older than 18 years who had a confirmed diagnosis of IBD, treated in a tertiary IBD centre (University Medical Centre Ljubljana, Slovenia), who started first-line treatment with UST, were included in this retrospective cross-sectional cohort study. Demographics, clinical characteristics of patients, treatment persistence of UST, clinical disease activity scores, C-reactive protein (CRP), faecal calprotectin (FC), UST concentrations, and endoscopic scores were collected retrospectively in medical files. We determined a Kaplan-Meier curve of treatment persistence. Results We included 71 patients, 11 of which had ulcerative colitis and 60 had Crohn’s disease. Persistence of treatment with UST in biologically naïve patients is 88% at one year (Figure 1). Clinical remission was achieved in 57.7% of patients. Biochemical remission was achieved in 77.6% (CRP<5mg/ml) and 71.1% (FC<100mg/kg) of patients. Endoscopic remission (Mayo endoscopic score <2 in ulcerative colitis or absence of ulcers in Crohn’s disease) was achieved in 50.0% of patients. There was no significant difference (P>0.05) between the median serum concentrations of UST in the group of patients who achieved remission (clinical (5.86μg/ml), biochemical (5.93μg/ml based on CRP, 5.63μg/ml based on FC) and endoscopic (7.14μg/ml)) and who did not achieve remission (5.31μg/ml, 3.25μg/ml, 4.47μg/ml and 4.64μg/ml, respectively). Conclusion Treatment of UST in biologically naïve IBD patients shows high treatment persistence with high biochemical and endoscopic remission rates. It appears that UST is more efficacious if used as first-line therapy compared to the use after other advanced therapies. Further prospective data are needed to confirm our findings.

P422 Prevalence of poor sleep quality assessed by wrist actigraphy and sleep questionnaire in Inflammatory Bowel Disease patients and its association with disease activity and quality of life

Abstract Background An association between active inflammatory bowel disease (IBD) and poor sleep quality has been proposed, however studies evaluating both subjective and objective assessment of disease activity and sleep quality are limited. Our study aimed to investigate the prevalence of poor sleep quality assessed by wrist actigraphy and sleep questionnaires in IBD patients and its association with disease activity and quality of life. Methods A prospective observational study was conducted in Siriraj Hospital, Mahidol University, Bangkok, Thailand from March 2021 to December 2022. Eligible IBD patients were categorized into remission, mild, and moderate-to-severe groups, based on standard clinical disease activity scores. Demographic data, inflammatory markers, inflammatory bowel disease questionnaire (IBDQ), sleep questionnaire (Pittsburgh Sleep Quality Index, PSQI), and seven-day sleep data from wrist actigraphy (Actiwatch®, CamNtech, Cambridge, United Kingdom) were obtained. The prevalence of sleep disorder and group comparison were analyzed. Results Eighty-four participants (40 Crohn’s disease, 44 UC), consisting of 60 remission, 16 mild, and 8 moderate-to-severe disease activity, were enrolled. Baseline demographic data were comparable between each group. Lower hemoglobin and albumin levels, and higher serum C-reactive protein, and fecal calprotectin levels were found in the more clinically active groups (table1). Fifty patients (60%) had poor sleep defined by PSQI > 5 with a trend to be higher in the group with active symptoms (57% in remission, 63% in mild, and 75% in moderate-to-severe group, p=0.31). Using actigraphy, the group with moderate-to-severe symptoms tended to have longer sleep latency, more frequently awake after sleep, and poorer sleep efficiency than the other groups (Figure 1). Patients with more severe disease activity had poorer quality of life with a mean IBDQ of 190 in remission, 159 in mild, and 134 in moderate-to-severe group (p<0.001). Conclusion Poor sleep quality is common among IBD patients, even those with clinical remission. Those with more severe symptoms had poorer sleep quality and worse quality of life. Sleep assessment and treatment should be integrated into IBD standard clinical care.

P881 Real-life vs trial access to biologic therapy differences: a 2019-2020 experience in an Italian tertiary IBD center

Abstract Background Inflammatory Bowel Diseases (IBD) are multifactorial diseases including Ulcerative Colitis (UC) and Crohn's Disease (CD). Up to 50% of IBD patients show a primary or secondary non-response to standard biological therapy. Hence, Randomized Clinical Trials (RCTs) represent a significant therapeutic opportunity for them. This study aims to describe the clinical characteristics of "trial IBD patients" vs. "real-life IBD". Methods We prospectively enrolled patients who started biologic therapy from August 2019 to August 2020. We divided patients into 3 sub-groups: (1) "real-life", patients treated according to clinical practice with biologic therapy that did not meet the inclusion and exclusion criteria for RCTs, (2) "real-life suitable for trial", patients potentially eligible in RCTs, but treated with standard of care, and (3) "trial", patients treated with drugs from 14 phases 2b and 3 studies actively recruiting in our center. Results We enrolled 134 patients (72 UC, 62 CD). "Real-life" patients were 41 in UC and 38 in CD, "real-life suitable for trial" were 6 in UC and 14 in CD, and "trial" were 25 in UC and 10 in CD. According to the existing inclusion and exclusion criteria, the study showed that 43% UC patients and 39% CD were suitable for enrollment in RCTs, however only 16% of CD were finally enrolled in RCTs. At the enrollment, patients were mainly excluded for topical therapy use, cancer, recent infections, low CDAI (<220) or SED-CD (<6), or presence of complication of diseases in CD or for limited extension of disease in UC. Both "trial" UC and CD patients presented more extraintestinal manifestations of disease, especially the articular ones (p<0,05) and a higher significant concomitant use of systemic corticosteroids (≤20mg/die (p < 0,05)). Percentages of patients treated previously with other biological drugs were superior in "trial" patients compared to "real-life" only in UC (88% vs 29%). We observed different baseline clinical disease activity scores in CD: the HBI of "real-life" patients was 4.8, and the HBI of "trial" patients was 9.1. In addition, patients enrolled in RCTs waited longer before accessing the proposed biological therapy. Conclusion This study highlights some differences between clinical practice and RCTs, particularly regarding criteria to start biologic therapy. Globally "trial patients" have more complex diseases that significantly impact their clinical status. Furthermore, RCTs use clinical scores (e.g. CDAI) as determinants for enrollment and decision-making, while endoscopy and radiological imaging are more widely used in clinical practice for decision making. These differences could cover the actual effectiveness of a new drug compared to the theoretical efficacy deriving from registration RCTs.

Safety and effectiveness of abatacept in juvenile idiopathic arthritis: results from the PRINTO/PRCSG registry.

The aim of this study was to report the interim 5-year safety and effectiveness of abatacept in patients with JIA in the PRINTO/PRCSG registry. The Abatacept JIA Registry (NCT01357668) is an ongoing observational study of children with JIA receiving abatacept; enrolment started in January 2013. Clinical sites enrolled patients with JIA starting or currently receiving abatacept. Eligible patients were assessed for safety (primary end point) and effectiveness over 10 years. Effectiveness was measured by clinical 10-joint Juvenile Arthritis Disease Activity Score (cJADAS10) in patients with JIA over 5 years. As-observed analysis is presented according to the Strengthening the Reporting of Observational Studies in Epidemiology (STROBE) guidelines. As of 31 March 2020, 587 patients were enrolled; 569 are included in this analysis (including 134 new users) with 1214.6 patient-years of safety data available. Over 5 years, the incidence rate (IR) per 100 patient-years of follow-up of serious adverse events was 5.52 (95% CI: 4.27, 7.01) and of events of special interest was 3.62 (95% CI: 2.63, 4.86), with 18 serious infections [IR 1.48 (95% CI: 0.88, 2.34)]. As early as month 3, 55.9% of patients achieved cJADAS10 low disease activity and inactive disease (20.3%, 72/354 and 35.6%, 126/354, respectively), sustained over 5 years. Disease activity measures improvement over 5 years across JIA categories. Abatacept was well tolerated in patients with JIA, with no new safety signals identified and with well-controlled disease activity, including some patients achieving inactive disease or remission. Clinicaltrials.gov, NCT01357668.

Real-World Clinical Equivalence of Generic and Branded Tofacitinib: A Prospective Longitudinal Cohort Study in Patients With Rheumatoid Arthritis

ObjectivesTo explore the clinical efficacy and safety of generic tofacitinib vs brand name tofacitinib in patients with rheumatoid arthritis (RA) in a single-center comparative study based on a prospective real-world cohort. MethodsPatients with RA receiving tofacitinib, either generic (Kelejia) or branded (Xeljanz), from March 2017, to December 31, 2022, were enrolled. The primary outcome was the simplified disease activity index (SDAI)–defined remission rate at month 6. Secondary outcomes included the rates of remission and low disease activity defined by other composite scores; European Alliance of Associations for Rheumatology response rate, and ultrasonic synovitis scores at months 1, 3, 6, and 12. Cost-effectiveness was investigated. Propensity score–based inverse probability of treatment weighting was adopted to reduce selection bias. ResultsA total of 204 patients were enrolled: 59 in the generic group and 145 in the branded group. An SDAI-defined remission was achieved in 41.1% and 39.2% of patients in the generic and branded groups, respectively, at month 6 (P=.85). Rates of remission and low disease activity achievement, changes in clinical disease activity scores, and power Doppler and gray scale synovitis scores at months 1, 3, 6, and 12 were comparable between the 2 groups. Similar proportions of patients in the groups achieved moderate/good response at months 1, 3, 6, and 12. Rates of drug retention and adverse effects were also similar in the 2 groups. Both Kelejia and Xeljanz were cost-effective, but Kelejia had a lower average cost-effectiveness ratio. ConclusionGeneric tofacitinib (Keljia) had equivalent clinical efficacy and safety and better cost-effectiveness compared with its originator (Xeljanz).

Using a collaborative learning health system approach to improve disease activity outcomes in children with juvenile idiopathic arthritis in the Pediatric Rheumatology Care and Outcomes Improvement Network.

The Pediatric Rheumatology Care and Outcomes Improvement Network (PR-COIN) is a North American learning health network focused on improving outcomes of children with juvenile idiopathic arthritis (JIA). JIA is a chronic autoimmune disease that can lead to morbidity related to persistent joint and ocular inflammation. PR-COIN has a shared patient registry that tracks twenty quality measures including ten outcome measures of which six are related to disease activity. The network's global aim, set in 2021, was to increase the percent of patients with oligoarticular or polyarticular JIA that had an inactive or low disease activity state from 76% to 80% by the end of 2023. Twenty-three hospitals participate in PR-COIN, with over 7,200 active patients with JIA. The disease activity outcome measures include active joint count, physician global assessment of disease activity, and measures related to validated composite disease activity scoring systems including inactive or low disease activity by the 10-joint clinical Juvenile Arthritis Disease Activity Score (cJADAS10), inactive or low disease activity by cJADAS10 at 6 months post-diagnosis, mean cJADAS10 score, and the American College of Rheumatology (ACR) provisional criteria for clinical inactive disease. Data is collated to measure network performance, which is displayed on run and control charts. Network-wide interventions have included pre-visit planning, shared decision making, self-management support, population health management, and utilizing a Treat to Target approach to care. Five outcome measures related to disease activity have demonstrated significant improvement over time. The percent of patients with inactive or low disease activity by cJADAS10 surpassed our goal with current network performance at 81%. Clinical inactive disease by ACR provisional criteria improved from 46% to 60%. The mean cJADAS10 score decreased from 4.3 to 2.6, and the mean active joint count declined from 1.5 to 0.7. Mean physician global assessment of disease activity significantly improved from 1 to 0.6. PR-COIN has shown significant improvement in disease activity metrics for patients with JIA. The network will continue to work on both site-specific and collaborative efforts to improve outcomes for children with JIA with attention to health equity, severity adjustment, and data quality.

MRI ankle in juvenile idiopathic arthritis and its comparison with clinical disease activity scores

Objectives: Juvenile idiopathic arthritis (JIA) is a common cause of morbidity and consequent disability among youth up to 16 years of age. Our study aims to image the ankle joints of JIA patients and correlate the findings with the clinical disease activity scores. Juvenile Arthritis Disease Activity Score (JADAS-27) is used by pediatricians to assess disease activity in JIA patients. There are four factors that determine this score – Global assessment of disease activity by the physician as well as patient/parent, joint count with active disease, and erythrocyte sedimentation rate (ESR). However, these clinical scores are dependent on the clinician’s experience and observation and, therefore, subjective. Material and Methods: Thirty patients of either sex with a clinical diagnosis of JIA as per the International League of Association for Rheumatology definition with clinically involved ankle joints were included in the study. Chronic arthritis due to other causes, such as infection, trauma, and so on, was excluded. All clinically diagnosed cases of JIA were subjected to magnetic resonance imaging (MRI) evaluation on Siemens Skyra 3 Tesla High-Resolution MR system using a dedicated coil for the ankle joint. Six sub-joints for each patient are evaluated, namely - The tibiotalar joint, subtalar joint, calcaneocuboid joint, calcaneonavicular joint, naviculocuneiform joint, and tarsometatarsal joint. Image analysis and scoring shall be done for each patient at each joint as per the Juvenile Arthritis MRI SCORE, consisting of the following parameters-synovial hypertrophy score, cartilage lesion score, bone erosion score, and bone marrow change score. Each of these scores is calculated individually based on the percentage of involved bone volume. All individual scores were summed to get the total MRI Score. The data collected were then statistically analyzed. Results: A positive correlation was sought between the JADAS-27 score and the total MRI score with the P-value being 0.03. A positive correlation was also found between the total MRI score and the ESR value of the patient (P - 0.02). Conclusion: MRI findings can be a potential marker of disease activity in JIA patients and these findings could also predict the prognosis accurately in these patients. The findings are objective and comparable to the aforementioned clinical scores.

A decade of progress in juvenile idiopathic arthritis treatments and outcomes in Canada: results from ReACCh-Out and the CAPRI registry.

To assess changes in juvenile idiopathic arthritis (JIA) treatments and outcomes in Canada, comparing 2005-2010 and 2017-2021 inception cohorts. Patients enrolled within three months of diagnosis in the Research in Arthritis in Canadian Children Emphasizing Outcomes (ReACCh-Out) and the Canadian Alliance of Pediatric Rheumatology Investigators Registry (CAPRI) cohorts were included. Cumulative incidences of drug starts and outcome attainment within 70 weeks of diagnosis were compared with Kaplan-Meier survival analysis and multivariable Cox regression. The 2005-2010 and 2017-2021 cohorts included 1128 and 721 patients, respectively. JIA category distribution and baseline clinical juvenile idiopathic arthritis disease activity (cJADAS10) scores at enrolment were comparable. By 70 weeks, 6% of patients (95% CI 5, 7) in the 2005-2010 and 26% (23, 30) in the 2017-2021 cohort had started a biologic DMARD (bDMARD), and 43% (40, 47) and 60% (56, 64) had started a conventional DMARD (cDMARD), respectively. Outcome attainment was 64% (61, 67) and 83% (80, 86) for inactive disease (Wallace criteria), 69% (66, 72) and 84% (81, 87) for minimally active disease (cJADAS10 criteria), 57% (54, 61) and 63% (59, 68) for pain control (<1/10), and 52% (47, 56) and 54% (48, 60) for good health-related quality of life (≥9/10). Although baseline disease characteristics were comparable in the 2005-2010 and 2017-2021 cohorts, cDMARD and bDMARD use increased with a concurrent increase in minimally active and inactive disease. Improvements in parent and patient-reported outcomes were smaller than improvements in disease activity.

E06 Does GGT level correlate with activity parameters in enthesitis-related arthritis?

Abstract Introduction Enthesitis-related arthritis (ERA) is one of the main subgroups of juvenile idiopathic arthritis. As in the adult population, monitoring of patients with ERA is based on clinical and laboratory evaluation. Some studies showed a correlation between Gamma-glutamyl transferase (GGT) levels and rheumatoid arthritis disease activity markers. Thus, we aimed to evaluate the correlation between GGT levels and ERA activity parameters. Methods This is a cross-sectional monocentric study including patients meeting the International League of Associations for Rheumatology (ILAR) 2001 criteria for ERA. We collected the demographic and clinical data. We assessed the ERA activity using clinical parameters including Patient Global Assessment (PGA), Visual Analogue Scale (VAS), Morning stiffness (MS), tender joint count (TJC), swollen joint count (SJC), active enthesitis count (AEC), clinical sacroiliitis, back mobility, and ocular involvement. We collected biological parameters such as the C-reactive protein (CRP) and the Erythrocyte sedimentation rate (ESR). We calculated the Juvenile spondyloarthritis disease activity (JSpADA). GGT level was measured on the same day. The correlation was evaluated using Spearman’s rho coefficient (r). r-value &amp;lt;0.3 was considered poor; between 0.3 and 0.39, moderate; between 0.4 and 0.69, strong; and &amp;gt;0.7 very strong. Results Thirty patients with a mean age of 15.1 ± 4.1 years, and a ratio-sex of 2.75 were included. The mean disease duration was 4.3 ± 3.2 years. The mean PGA and the mean VAS were 6.2 ± 7.1 and 8.9 ± 2.9 respectively. Mean TJC, mean SJC, and mean AEC were 1.53 ± 2.8, 0.18 ± 0.47, and 1.2 ± 1.6 respectively. Clinical sacroiliitis was found in eight patients, limited back mobility in six, and uveitis in three patients. The mean CRP and ESR were 15.1 ± 22.7 mg/l and 23.8 ± 21.2 mm, respectively. The mean JSpADA was 2.6 ± 1.7. Mean GGT was 14.3 ± 6.6 mg/l. A strong correlation was noted between the GGT level and both CRP (r = 0.504, P = 0.05) and ESR (r = 0.601, P = 0.01). However, no correlation was noted between GGT level and PGA (r = 0.107, P = 0.6), VAS (r = 0.03, P = 0.9), SJC (r = -0.17, P = 0.368), nor JSpADA (r = 0.323, P = 0.87). Conclusion Our results suggest a strong correlation between GGT level and biological ERA activity parameters, unlike clinical parameters and disease activity scores. More studies are needed to confirm these findings. Ethics The authors have no conflicts of interest to declare.

Feasibility of Conducting Comparative Effectiveness Research and Validation of a Clinical Disease Activity Score for Chronic Nonbacterial Osteomyelitis.

Prospective comparative effectiveness research (CER) in chronic nonbacterial osteomyelitis (CNO) is lacking. Our objectives were to (1) determine the use and safety of each consensus treatment plan (CTP) regimen for CNO, (2) assess the feasibility of using the Chronic Nonbacterial Osteomyelitis International Registry (CHOIR) data for CER, and (3) develop and validate a CNO clinical disease activity score (CDAS) using CHOIR. Consenting children or young adults with CNO were enrolled into CHOIR. Demographic, clinical, and imaging data were prospectively collected. The CNO CDAS was developed through a Delphi survey and nominal group technique. External validation surveys were administered to CHOIR participants. One hundred forty (78.2%) CHOIR participants enrolled between August 2018 and September 2020 received at least 1 CTP regimen. Baseline characteristics from different CTP groups were well matched. Patient pain, patient global assessment, and clinical CNO lesion count were key variables included in the CNO CDAS. The CDAS showed a strong correlation with patient/parent report of difficulty using a limb, back, or jaw and patient/parent report of disease severity, but a weak correlation with patient/parent report of fatigue, sadness, and worry. The change in CDAS was significant in patients reporting disease worsening or improvement (P < 0.001). The CDAS significantly decreased after initiating second-line treatments from median 12.0 (IQR 8.0-15.5) to 5.0 (IQR 3.0-12.0; P = 0.002). Although second-line treatments were well tolerated, psoriasis was the most common adverse event. The CNO CDAS was developed and validated for disease monitoring and assessment of treatment effectiveness. CHOIR provided a comprehensive framework for future CER.

Fatigue in Patients with Newly Diagnosed Inflammatory Bowel Disease: Results from a Prospective Inception Cohort, the IBSEN III Study.

Although fatigue is common in inflammatory bowel disease [IBD], its pathogenesis remains unclear. This study aimed to determine the prevalence of fatigue and its associated factors in a cohort of patients newly diagnosed with IBD. Patients ≥18 years old were recruited from the Inflammatory Bowel Disease South-Eastern Norway [IBSEN III] study, a population-based, observational inception cohort. Fatigue was assessed using the Fatigue Questionnaire and compared with data from a Norwegian general population. Univariate and multivariate linear and logistic regression analyses were performed to evaluate the associations of total fatigue [TF; continuous score] and substantial fatigue [SF; dichotomized score ≥4] with sociodemographic, clinical, endoscopic, laboratory, and other relevant patient data. In total, 983/1509 [65.1%] patients with complete fatigue data were included (ulcerative colitis [UC], 68.2%; Crohn's disease [CD], 31.8%). The prevalence of SF was higher in CD [69.6%] compared with UC [60.2%] [p < 0.01], and in both diagnoses when compared to the general population [p < 0.001]. In multivariate analyses, depressive symptoms, pain intensity, and sleep disturbances were associated with increased TF for both diagnoses. In addition, increased clinical disease activity and Mayo endoscopic score were significantly associated with TF in UC, whereas all disease-related variables were insignificant in CD. Similar findings were observed for SF, except regarding the Mayo endoscopic score. SF affects approximately two-thirds of patients newly diagnosed with IBD. Fatigue was associated with depressive symptoms, sleep disturbances, and increased pain intensity in both diagnoses, while clinical and endoscopic activity were associated factors only in UC.