Development and initial validation of parent and child versions of the Juvenile Arthritis Disease Activity Score.

OBJECTIVE: To develop parent- and child-centered versions of the Juvenile Arthritis Disease Activity Score (JADAS) and to provide preliminary evidence of their validity. METHODS: Validation analyses were conducted on two large multinational datasets of patients with juvenile idiopathic arthritis (JIA) and included assessment of construct validity, internal consistency and structure, discriminative validity, responsiveness to change, and predictive validity. RESULTS: The parJADAS and patJADAS include four parent/patient-reported outcomes, each measured on a 0-10 scale: assessment of overall disease activity; rating of pain intensity; assessment of activity of joint disease; duration of morning stiffness. Both scores are calculated as the simple linear sum of the scores of their 4 components, which yields for both of them a global score of 0-40. The parJADAS and patJADAS demonstrated good construct validity, yielding high correlations with other JIA composite disease activity measures and moderate correlations with physician global rating and joint counts. Internal consistency was satisfactory, with Cronbach' s alpha > 0.80, and exploratory factor analysis showed that both indices are monodimensional. Both instruments discriminated well between different disease states, with discriminative ability being not affected by the presence of damage, proved able to predict important disease outcomes, and showed fair responsiveness to clinically important change, with standardized response mean of 0.71. CONCLUSION: Both parJADAS and patJADAS were found to possess good measurement properties and to serve as surrogate of physicians' evaluations. Regular home completion of the two instruments through digital technologies offers a suitable and pragmatic approach to deliver remote symptom monitoring and telehealth.

Defining Criteria for Disease Activity States in Systemic Juvenile Idiopathic Arthritis Based on the Systemic Juvenile Arthritis Disease Activity Score.

OBJECTIVE: Our objective was to develop and validate cutoff values in the systemic Juvenile Arthritis Disease Activity Score 10 (sJADAS10) that distinguish the states of inactive disease (ID), minimal disease activity (MDA), moderate disease activity (MoDA), and high disease activity (HDA) in children with systemic juvenile idiopathic arthritis, based on subjective disease state assessment by the treating pediatric rheumatologist. METHODS: The cutoff definition cohort was composed of 400 patients enrolled at 30 pediatric rheumatology centers in 11 countries. Using the subjective physician rating as an external criterion, six methods were applied to identify the cutoffs: mapping, calculation of percentiles of cumulative score distribution, the Youden index, 90% specificity, maximum agreement, and receiver operating characteristic curve analysis. Sixty percent of the patients were assigned to the definition cohort, and 40% were assigned to the validation cohort. Cutoff validation was conducted by assessing discriminative ability. RESULTS: The sJADAS10 cutoffs that separated ID from MDA, MDA from MoDA, and MoDA from HDA were ≤2.9, ≤10, and >20.6, respectively. The cutoffs discriminated strongly among different levels of pain, between patients with and without morning stiffness, and among patients whose parents judged their disease status as remission or persistent activity or flare or were satisfied or not satisfied with current illness outcome. CONCLUSION: The sJADAS cutoffs revealed good metrologic properties in both definition and validation cohorts and are therefore suitable for use in clinical trials and routine practice.

Defining criteria for disease activity states in juvenile dermatomyositis based on the Juvenile Dermatomyositis Activity Index.

OBJECTIVES: To develop and validate the cut-offs in the Juvenile DermatoMyositis Activity Index (JDMAI) to distinguish the states of inactive disease (ID), low disease activity (LDA), moderate disease activity (MDA) and high disease activity (HDA) in children with juvenile dermatomyositis (JDM). METHODS: For cut-off definition, data from 139 patients included in a randomised clinical trial were used. Among the six versions of the JDMAI, JDMA1 (score range 0-40) and JDMAI2 (score range 0-39) were selected. Optimal cut-offs were determined against external criteria by calculating different percentiles of score distribution and through receiver operating characteristic curve analysis. External criteria included the modified Pediatric Rheumatology International Trials Organization (PRINTO) criteria for clinically ID in JDM (for ID) and PRINTO levels of improvement in the clinical trial (for LDA and HDA). MDA cut-offs were set at the score interval between LDA and HDA cut-offs. Cut-off validation was conducted by assessing construct and discriminative ability in two cohorts including a total of 488 JDM patients. RESULTS: The calculated JDMAI1 cut-offs were ≤2.4 for ID, ≤6.6 for LDA, 6.7-11 for MDA and >11 for HDA. The calculated JDMAI2 cut-offs were ≤5.2 for ID, ≤8.5 for LDA, 8.6-11.3 for MDA and >11.3 for HDA. The cut-offs discriminated strongly among disease activity states defined subjectively by caring physicians and parents, parents' satisfaction or non-satisfaction with illness outcome, levels of pain, fatigue, physical functional impairment and physical well-being. CONCLUSIONS: Both JDMAI1 and JDMAI2 cut-offs revealed good metrologic properties in validation analyses and are, therefore, suited for application in clinical practice and research.

Clinical features, treatment and outcomes of Italian children with enthesitis-related arthritis and juvenile psoriatic arthritis: a cross-sectional cohort study.

OBJECTIVES: Limited information is available on the clinical features, treatment modalities and outcomes of the juvenile idiopathic arthritis (JIA) categories of enthesitis-related arthritis (ERA) and juvenile psoriatic arthritis (JPsA). This study was aimed to describe the characteristics of Italian children with ERA and JPsA and to compare them with those of patients with the other categories of JIA. METHODS: Patients were part of a multinational sample included in a study aimed to investigate the prevalence of disease categories, treatment approaches, and disease status in patients from across different geographical areas (EPOCA Study). All patients underwent a retrospective assessment, based on the review of clinical chart, and a cross-sectional evaluation, which included assessment of physician- and parent-reported outcomes and laboratory tests, and recording of ongoing therapies. RESULTS: Of the 9081 children with JIA enrolled in the EPOCA Study, 1300 were recruited at 18 paediatric rheumatology centres in Italy. 45 (3.5%) had ERA and 49 (3.8%) had JPsA. Several remarkable differences in demographic features and frequency of articular and extra-articular manifestations, disease damage, impairment in physical function and health-related quality of life, school-related problems, comorbidities, and ongoing treatments were observed between ERA and JPsA and the other JIA categories. CONCLUSIONS: We described the characteristics of Italian children with ERA and JPsA and highlighted their peculiarities and their differences from the other JIA subsets. These data provide useful insights for future revisions of JIA classification and a benchmarking against which the features from other cohorts may be compared.

Treat-to-Target in Pediatric Rheumatic Diseases.

PURPOSE OF REVIEW: To summarize the current evidence on the adoption of the treat-to-target (T2T) strategy in pediatric rheumatic diseases (PRD). RECENT FINDINGS: The recent advances in the management of PRD have markedly increased the ability to achieve disease remission. Complete disease quiescence is regarded as the ideal therapeutic goal because its attainment leads to lesser long-term damage and physical disability, and to optimization of quality of life. Studies in adult rheumatic diseases have shown that patient outcomes are improved if complete suppression of the inflammatory process is aimed for by frequent adjustments of therapy according to quantitative indices. This approach, which underlies the T2T concept, has been applied in strategic trials in rheumatoid arthritis (RA). Furthermore, recommendations for the T2T have been issued for RA and other adult rheumatic diseases. There is currently a growing interest for the introduction of T2T in PRD, and recommendations for treating juvenile idiopathic arthritis (JIA) to target were promulgated. A similar initiative has been undertaken for childhood-onset systemic lupus erythematosus. Preliminary therapeutic studies have explored the T2T design in JIA. The T2T strategy is a modern therapeutic approach that holds the promise of improving the outcomes in patients with PRD.

Juvenile Psoriatic Arthritis: Myth or Reality? An Unending Debate.

Juvenile psoriatic arthritis (JPsA) accounts for 1-7% of all cases of juvenile idiopathic arthritis (JIA) and its definition has been a matter of controversy among pediatric rheumatologists for many years. The traditional attribution of JPsA to the spondyloarthropathy group was challenged in the early 1990s, whereas the recent demonstrations of its heterogenous nature have led to questions about its identification as a distinct category in JIA classification. It has been shown that children with the phenotype of JPsA can be divided in two subgroups, one presenting with the features of early-onset ANA-positive JIA, and another that belongs to the spectrum of spondyloarthropathies. The few studies that have compared the clinical characteristics and genetic determinants of JPsA with those of the other JIA categories have obtained contrasting findings. The debate on the categorization of JPsA as a distinct entity within JIA classification is still ongoing and has prompted the revision of its current classification.

How Variable Is the Volar Subcutaneous Tissue of the Digits on B-Mode and Color Doppler Ultrasound in Non-Psoriatic Individuals and Could It Be Included in a Dactylitis Score?

BACKGROUND: Digital subcutaneous tissue (SCT) changes are involved in dactylitis, a hallmark feature of psoriatic arthritis (PsA). There are no studies on the ultrasound (US) characteristics of the digital SCT in the general population. OBJECTIVES: To investigate the variability in US-measured thickness (TH) and color Doppler (CD)-detected blood flow of the SCT of the volar aspects of the fingers in a non-psoriatic population and to investigate the impact of the scanning method and demographics and clinical features on these measurements. METHODS: SCT TH and semiquantitative (SQD) and quantitative (QD) Doppler signals were measured in the bilateral second finger at the proximal and middle phalanges in 81 non-psoriatic volunteers [49 female, 32 men; 18-78 years]. Two scanning methods with and without (thick gel layer interposition) probe-skin contact were used. Demographics and clinical features were collected. RESULTS: There was high variability of SCT TH and Doppler measurements between individuals. All US measurements obtained without probe-skin contact were significantly greater than their corresponding measurements obtained with the probe contacting the skin (p < 0.001). SCT TH was positively related to dominant hand, age, masculine gender, weight, height, body mass index, and alcohol consumption while Doppler measurements were positively related to age and non-dominant hand. CONCLUSIONS: US-measured SCT thickness and Doppler-detected SCT blood flow of the volar aspect of the fingers seem to be highly variable in the non-psoriatic population as well as highly dependent on the US scanning method. This variability is of utmost importance for assessing dactylitis in PsA.

Vitamin D, an inflammatory activity marker for spondyloarthritis?

In this study, a relationship between low levels of vitamin D and other markers of inflammation is observed in patients with spondyloarthritis. Therefore, insufficient levels of vitamin D could be used as a marker of inflammation, so they would help to have a better follow-up of the patient. PURPOSE: The aim of this study is to determine the association between 25-hydroxyvitamin D deficiency and level of disease activity in patients suffering from spondyloarthritis. METHODOLOGY: This study is an observational, descriptive and transversal study. A retrospective review of patients with spondyloarthritis under treatment at the Rheumatology Department of Hospital General Universitario de Ciudad Real between September 2016 and September 2018 is under consideration. The association between 25-hydroxyvitamin D deficiency and levels of disease activity was calculated using odds ratio (OR) with a 95% confidence level. RESULTS: Initial study developments are reported. A group of 147 patients was analyzed. The average disease activity was 4.3 (according to BASDAI) and 11.4 (according to DAPSA). Twenty-eight percent of patients showed increased levels of acute-phase reactants, and 82% exhibited 25-hydroxyvitamin D deficiency/insufficiency. Analysis of the association between vitamin D deficiency/insufficiency and BASDAI/DAPSA activity degree revealed an OR of 7.9 (CI95%: 2.92-21.23, p = < 0.0001). CONCLUSION: In this study, an association was found between those patients with spondyloarthritis who had a 25-hydroxyvitamin D deficiency and disease activity. Despite these results, a 1-year follow-up of these patients using two or more measurements of activity and 25-hydroxyvitamin D levels would be needed to corroborate these data.

Radioisotope Synoviorthesis in the Control of Refractory Synovitis in Castilla-La Mancha. A 10-year Experience. / La sinoviortesis radioisotópica en el control de la sinovitis refractaria en Castilla La-Mancha. Una experiencia de 10 años.

Our hospital is the nuclear medicine referral hospital for radioisotopic synoviorthesis for all of Castilla-La Mancha. OBJECTIVE: To describe the experience in the performance of radioisotopic synoviorthesis for arthritis refractory to other treatments in our hospital. METHODOLOGY: Observational, descriptive and cross-sectional study protocolised through the review of the database of radioisotopic synoviorthesis performed between 2007 and 2017. Previous clinical data were collected (age, sex, pathology, previous treatments, previous infiltration and affected joint), and progress at 6 months after administering the isotope. An Excel database was created for a frequency analysis with SPSS 21. RESULTS: 30 radiosynovitis interventions were performed. The most frequent pathologies in this order were: pigmented villonodular synovitis (40%), rheumatoid arthritis (23.3%), spondyloarthritis (13.3%), osteoarthritis (10%) and nonspecific arthritis (6.7%), followed by systemic lupus erythematosus and gout. After 6 months, 56.7% of the patients improved compared to 36.7% who remained the same. Likewise, none of them presented complications related to the procedure. Six point six percent of the patients were lost to follow-up. DISCUSSION AND CONCLUSIONS: In patients with episodes of recurrent arthritis with associated joint effusion in one or two joints, refractory to systemic treatments, to local infiltrations with corticosteroids and for patients for whom other treatments may be contraindicated, we must consider the possibility of performing an isotope radiosinoviortesis, as it is a simple, safe technique with a success rate of more than 50%.

Rheumatic syndromes and occult tumor: 10-year experience in a teaching hospital. / Síndromes reumáticos y tumor oculto, experiencia de 10 años en un hospital universitario.

OBJECTIVE: To describe the different clinical characteristics of patients admitted to the Rheumatology Department due to rheumatic manifestations as the first expression of an unknown malignant process. PATIENTS AND METHODS: Retrospective and descriptive observational study involving the review of the medical records of those admitted to rheumatology in the University Hospital of Ciudad Real between January 2007 and August 2017 for initial rheumatic manifestations with a suspicion at discharge of an unknown tumor. RESULTS: In all, 64 patients were identified from more than 500 admissions. The most common rheumatic manifestations were inflammatory low back pain, polyarthralgia, hip pain, thoracic spine pain, cervical pain, polyarthritis and polymyalgia rheumatica. Forty-four percent had low hemoglobin, 70% had elevation of acute-phase reactants, 62% had abnormal tumor markers, 76% had metastatic lesions. In 20% the primary tumor was of pulmonary origin and only 26.56% received palliative treatment; 64% died. DISCUSSION: It is important to consider the possibility of an underlying malignant process in the differential diagnosis since its early identification can be determinant for prognosis.