Rheumatology International Research Articles

SP0110 European Initiative in Pediatric Rheumatology. the Example of the Paediatric Rheumatology International Trials Organisation (PRINTO)

The existence of large networks such as the Pediatric Rheumatology Collaborative Study Group (PRCSG) and the Paediatric Rheumatology International Trials Organization (PRINTO), the Pediatric Rheumatology European Society (PRES) and more...

SP0142 Developing Eular Recommendations for the Use of Imaging in Spondyloarthritis Clinical Practice: Background and Process

Reflecting the perceived need for developing evidence-based recommendations on the use of imaging of the joints in the clinical management of spondyloarthritis (SpA) a European League Against Rheumatism (EULAR) convened...

Disease activity and prognostic factors in juvenile dermatomyositis: a long-term follow-up study applying the Paediatric Rheumatology International Trials Organization criteria for inactive disease and the myositis disease activity assessment tool

The aims of this study were to examine disease activity by the Paediatric Rheumatology International Trials Organization (PRINTO) criteria for inactive disease and the Myositis Disease Activity Assessment Tool (MDAAT) in JDM patients after long-term follow-up and to identify predictors of these outcomes. A retrospective inception cohort of 59 patients diagnosed with JDM was clinically examined in a cross-sectional study a median of 16.8 years (range 2.0-38.1) after symptom onset. Patients were divided by the PRINTO criteria into clinically inactive and active disease. Disease activity was also measured by MDAAT and other validated tools. Medical records were reviewed for early disease variables and medication. By the PRINTO criteria, 31/59 (51%) patients were active and 29/59 (49%) were inactive. By MDAAT, 43/59 (73%) of the patients had measurable disease activity, most commonly found in the skin (59%) and skeletal (27%) systems. MDAAT showed moderate to strong correlations with other disease activity measures (rsp 0.39-0.87, P < 0.05) except for muscle enzymes. Active patients had higher disease activity than inactive patients measured by MDAAT (P < 0.001) and other disease characteristics (all P ≤ 0.002) except for patients' global assessment of disease activity. After controlling for gender and follow-up time, calcinosis during disease-course predicted high MDAAT, age<9 years at diagnosis predicted active disease and organ damage present 6-12 months post diagnosis predicted both outcomes. After 16.8 years, 51-73% of JDM patients had active disease. Disease activity by the PRINTO criteria and MDAAT were moderately to highly associated with most other disease characteristics and was predicted by early damage.

A144: Resident's Guide to Rheumatology Guide Mobile Application: An International Needs Assessment

Background/Purpose:“A Resident's Guide To Pediatric Rheumatology” (the Guide) is a widely accepted resource for pediatric rheumatologists and trainees. In preliminary assessments, uptake of the Guide was broader than intended and it was used by trainees to help with clinical decision‐making, learning and teaching. Users of the Guide suggested that it be developed into a mobile application (app)The Technology Acceptance Model (TAM) provides a framework to assess the perceived usefulness and ease of use of a tool to predict future acceptance and use.Objectives: To determine the International demand amongst pediatric professionals and current trainees for a mobile app format of the Guide. To determine user preferred features, functions, and format to be included in a mobile app using the TAM.Methods:An electronic survey was developed and distributed to pediatric residents at SickKids hospital and to both faculty and trainee members of the international Pediatric Rheumatology list server. The survey included respondent demographics, perceived usefulness, perceived ease of use, and behavioural intention to use the app based on the TAM. Data were analyzed using descriptive statistics.Results:The survey was distributed to 75 pediatric residents and 1132 members of the Pediatric Rheumatology listserver and 135 (12% response rate) completed the survey. The majority of respondents were rheumatologists (53%), while the remainder consisted of Fellows (17%), Pediatric Residents (16%), and other allied health professionals (5%). 93% owned a smartphone and 58% owned a tablet. Most had medically related apps (75%) compared to e‐books (38%), but had similar use for each—Mone to several times per week for 1–15 minutes each time on average.The most useful features of an app would be clinical pictures (e.g. skin rashes), radiology images (e.g. joint x‐rays), and definitions of key terms. Least useful features were games and multiple‐choice questions. Additional features included a searchable index and links to journal articles.Looking at the TAM, the vast majority of respondents thought that the mobile app would enhance trainees' learning and teaching effectiveness. Greater than 80% of respondents consistently supported its perceived ease of use. 55% stated that they were likely to use the app often.86% felt it was important for the app to be developed. If the app was not available for free, a majority (43%) of respondents were willing to pay for the app with a most willing to pay up to $5.00, and 10% willing to pay up to $10 for access to the app.Conclusion:Development of the Guide app was well supported with adding features such as clinical photographs, radiology images, definitions and searchable index. TAM showed the intention to use the app in the future will be most determined by the perceived ease of use which was consistently high in the survey. Interestingly, users were willing to pay for the app if it was not free.Future steps include a qualitative study ultilizing focus groups to assess the perceived functionality, usability, facilitators and barriers in using the Guide app prototype to create the most targeted, user friendly app.

A111‐a: Setting Up of Japanese Online Registry of Pediatric Rheumatic Disease

Background/Purpose:Performing quality clinical and translational research in pediatric rheumatic diseases (eg. Juvenile Idiopathic Arthritis (JIA), Child onset Systemic Lupus Erythematosus (cSLE) and Juvenile Dermatomyositis (JDM)) had been difficult due the rarity of these diseases.Methods:Professor Takei, the former chairman of Pediatric Rheumatology Association of Japan (PRAJ), had ordered his working group to set up a new registry system in 2012. We, working group, have set up Japanese online registry for pediatric rheumatic disease in 2014 and have named it as Pediatric Rheumatology International Collaboration Unit Registry (PRICURE).Results:Under this registry, PRAJ will collect basic information (e.g. age of onset, sex, symptoms, major complication, main treatment) about these diseases (JIA, SLE, JDM, Sjogren syndrome, Mixed Connective Tissue Disease, Systemic Sclerosis, Behcet's disease, Vasculitis, Autoinflammatory syndromes, Fibromyalgia).Conclusion:We had set up new registry of pediatric rheumatic diseases. We will show initial data from this registry here.

Clinical and laboratory profile of childhood polyarteritis nodosa in a Bangladeshi tertiary hospital

Polyarteritis nodosa in children is a rare necrotizing vasculitis affecting mainly small and medium-size arteries. To describe the different clinical patterns and laboratory profiles of polyarteritis nodosa patients in a tertiary care hospital. This was a retrospective cohort study carried out in the Department of Paediatrics, Bangabandhu Sheikh Mujib Medical University, Dhaka, Bangladesh during the period January 2007 to December 2012. A total of 13 patients fulfilling the European League Against Rheumatism/Paediatric Rheumatology International Trial Organization/Paediatric Rheumatology European Society (EULAR/PRINTO/PRES) classification criteria were enrolled in this study. Data was collected via a predesigned questionnaire. Age range was 3-12 years, male : female ratio was 9 : 4. The duration of symptoms was 2-16 weeks. All the children had fever, anorexia and generalized weakness. Subcutaneous nodules were present in 77% of cases followed by arthritis and rash (69%), muscle pain (54%) and abdominal pain (38%). Impaired peripheral pulses were present in 54%, ulceration and gangrene was present in 31% and auto-amputation was present in 15% of cases. All the patients had high erythrocyte sedimentation rates followed by neutrophilic leukocytosis and thrombocytosis (85% and 62%). Skin biopsy was positive in 77% of cases and angiographic abnormalities were present in 23% of cases. Most clinical and laboratory profiles of polyarteritis nodosa in our center, such as age, sex, fever, rash, arthritis and abdominal pain, were mostly similar to other reports; however some late cases were found in this series with complications such as gangrene and auto-amputation.

Analysis of randomized controlled trials in Rheumatology International from 1981 to 2012: methodological assessment

The aim of the study is to assess the methodological quality of randomized controlled trials (RCTs) published in Rheumatology International (RI) by using three types of analytical tools. MEDLINE was used to extract RCTs from original articles published in the RI from 1981 (vol. 1) to 2012 (vol. 32). The relationship between the number of articles and RCTs with time and that between various factors and the quality of RCTs were analyzed. To analyze the methodological quality of the RCTs, the time period was divided into several sections and three tools were applied (e.g., the Jadad scale, van Tulder scale, and Cochrane Collaboration Risk of Bias Tool). The number of RCTs published gradually increased with time significantly (p < 0.001). The differences in RCT quality scores by each method in the publication years evaluated were not statistically significant, but RCTs that included descriptions of allocation concealment methods had received institutional review board (IRB) approval, and that conducted in the multicenter had significantly higher-quality scores than other studies. In conclusion, although the number of RCTs published in RI since its publishing in 1981 has increased with time, but no qualitative improvement of RCT was observed over time. It is necessary to improve the reporting of concealment of allocation, generation of randomization sequences, design of blinded studies, and obtaining IRB approval, all of which are criteria of high-quality RCTs.

Diagnostic and classification criteria of Takayasu arteritis

Takayasu arteritis (TA) is a chronic large vessel vasculitis that affects aorta, its main branches and pulmonary arteries. The inflammatory process results in stenosis, occlusion, dilation or aneurysm formation in the arterial wall. TA has been described in different parts of the world and affects predominantly young individuals (<50 years of age). Patients with TA may present constitutional symptoms, vascular pain (e.g. carotidynia) and typical features such as limb claudication, decreased or absent peripheral pulses, vascular bruits, hypertension, and reduction or discrepancies in blood pressure between arms. A proper diagnosis of TA is an important issue since delays may result in significant morbidity. The definition of TA was included in the 1994 and 2012 Chapel Hill Consensus Conference and TA was categorized as a large vessel vasculitis. The first diagnostic criteria for TA were developed by Ishikawa in 1988 and modified by Sharma et al., in 1995. Two sets of classification criteria were developed for TA to include patients in epidemiologic studies and clinical trials: the 1990 ACR Classification Criteria for TA and the Classification Criteria for childhood TA proposed by the European League Against Rheumatism (EULAR), the Pediatric Rheumatology European Society (PRES) and by the Pediatric Rheumatology International Trials Organization (PRINTO) to be used for patients younger than 18 years. The Diagnostic and Classification Criteria in Vasculitis Study (DCVAS) is an international effort that is under way to develop a single classification system and a validated set of diagnostic criteria for systemic vasculitides using data-driven methods.

Age of onset as a risk factor of renal involvement in Henoch-Schönlein purpura

BackgroundHenoch-Schönlein purpura (HSP) is the most common vasculitis in children, characterized by triad of symptoms; palpable purpura without thrombocytopenia, abdominal pain, and arthritis. Renal involvement often occur in children with HSP. No data on the renal involvement of children with HSP in Indonesia, especially West Java.ObjectiveTo evaluate renal involvement in children with HSP.MethodsRetrospective study was conducted in children with HSP in Department of Child Health, Hasan Sadikin Hospital, from 2006 to 2011. Characteristics and clinical manifestations was reviewed from medical record. HSP was diagnosed by American College of Rheumatology 1990 criteria or European League Against Rheumatism/Pediatric Rheumatology International Trials Organization/Pediatric Rheumatology European Society 2008.ResultsThere were 128 patients, consisting of 82 male (64.9%) and 46 female (35.1%) with ratio 1.8:1. Mean age was 7.9 ± 2.9 years old which range from 6 month to 15 years. Peak morbidity was between 5-10 years old. Prevalence of HSP in Hasan Sadikin Hospital tend to raise from 2.7/100,000 in 2008 to 5.2/100,000 in 2010. In most patients (71%) purpura was the first symptom. Seventy-one patients (44.5%) had arthritis and 89 patients (69.5%) had abdominal pain, while renal involvement was in 28 patients (21.8%). Gastrointestinal manifestations tend to manifest in patients less than 5 years old (p = 0.267), while renal involvement tend to manifest in age group 11-15 years old (p = 0.015) with odds ratio 3.1 (95% confidence interval, 1.2-8.1).ConclusionRenal involvement in children with HSP is more common in age group 11 to 15 years old.

PReS-FINAL-2329: Validation of the autoinflammatory activity index (AIDAI)

With the increasing potential for targeted therapies in autoinflammatory diseases, there is the need for validated and standardized assessment tools which can be used to evaluate the level of disease activity and response to therapy. An international collaboration, initiated by Assistance Publique-Hopitaux de Paris (APHP) in association with the Paediatric Rheumatology International Trials Organization (PRINTO at http://www.printo.it) and supported by the EUROFEVER and EUROTRAPS networks, has previously designed the content and the preliminary scoring of an Auto-Inflammatory Disease Activity Index (AIDAI)

Current state of musculoskeletal ultrasound in paediatric rheumatology: results of an international survey

The aim of this study was to investigate the current use of musculoskeletal US (MSUS) and the most relevant areas of interest for this imaging modality in paediatric rheumatology. A questionnaire was developed by the paediatric subgroup of the OMERACT US task force and e-mailed to the members of the main international paediatric rheumatology networks and societies. Responses were entered in an electronic database. Results were analysed quantitatively or summarized qualitatively in the case of open questions. The overall response rate was 36% (262/719). The use of MSUS varied among members of the various networks/societies. MSUS was considered of high relevance for improvement of diagnostic skills, for the guidance of joint injections and for the assessment of specific joints, namely the hip, ankle, midfoot and wrist. It was considered useful for early detection of synovitis and in determining disease activity and disease remission. Although at present MSUS is not widely used by paediatric rheumatologists, there is considerable interest in this imaging technology among members of the international networks. The results of this survey suggest that the next objective in the research agenda should be the standardization of the assessment of joints in healthy children. This will then help differentiate pathological (i.e. synovitic) joints from normal joints. The initial target joints should be the hip, ankle, midfoot and wrist. MSUS training focused on the assessment of paediatric patients might be very important in implementing the use of this technique in clinical practice and research.

OR7-004 – Validation of AIDAI score

With the increasing potential for targeted therapies in autoinflammatory diseases, there is the need for validated and standardized assessment tools which can be used to evaluate the level of disease activity and response to therapy. An international collaboration, initiated by Assistance Publique-Hopitaux de Paris (APHP) in association with the Paediatric Rheumatology International Trials Organization (PRINTO at http://www.printo.it) and supported by the EUROFEVER and EUROTRAPS networks, has previously designed the content and the preliminary scoring of an Auto-Inflammatory Disease Activity Index (AIDAI).

Treating rheumatoid arthritis to target: recommendations assessment questionnaire in Korea

Treat rheumatoid arthritis (RA) to target (T2T) is an international initiative to provide the rheumatology community with clear direction on treatment targets for RA. We performed a nationwide survey in Korea among rheumatologists to measure the levels of agreement with international T2T recommendations and to assess their practical application in Korea. A questionnaire was administered to 111 physicians. Responses were assessed using a 10-point Likert scale for the level of agreement with each of 10 recommendations and a 4-point Likert scale for the degree to which each recommendation was being applied in current daily practice. Respondents were also asked whether these recommendations would result in a change in their practice. This report outlines the consensus reached for T2T in Korea and compares those results with data obtained internationally. Agreement with T2T recommendations was high with average response scores above 8.3. The majority of respondents indicated they applied the recommendations "always" and "very often" in daily practice. More than half of the participants not currently applying these recommendations were willing to change their practice, but the percentage of Korean physicians willing to change was consistently more than the international average. The results of this survey of T2T recommendations in Korean rheumatologists revealed a good correlation with the views of the international rheumatology community. These results could be utilized to define key issues for better disease control in daily practice and used as a reference to improve the treatment environment.

Calculation skills for nurses: psoriatic arthritis

Nurse PrescribingVol. 11, No. 9 Calculation SkillsCalculation skills for nurses: psoriatic arthritisDilyse NuttallDilyse NuttallSearch for more papers by this authorDilyse NuttallPublished Online:30 Sep 2013https://doi.org/10.12968/npre.2013.11.9.430AboutSectionsView articleView Full TextPDF/EPUB ToolsAdd to favoritesDownload CitationsTrack CitationsPermissions ShareShare onFacebookTwitterLinked InEmail View article References Joint Formulary Committee (2012) British National Formulary 63. British Medical Association and Royal Pharmaceutical Society of Great Britain, London Google Scholar National Institute for Health and Clinical Excellence (2010) NICE technology appraisal guidance 199. Etanercept, infliximab and adalimumab for the treatment of psoriatic arthritis. http://www.niceorg.uk/nicemedia/live/13110/50422/50422.pdf. (accessed 18 September 2012) Google Scholar FiguresReferencesRelatedDetailsCited byBenefit of health education by a training nurse in patients with axial and/or peripheral psoriatic arthritis: A systematic literature review20 August 2016 | Rheumatology International, Vol. 36, No. 11 1 September 2013Volume 11Issue 9ISSN (print): 1479-9189ISSN (online): 2052-2924 Metrics History Published online 30 September 2013 Published in print 1 September 2013 Information© MA Healthcare LimitedPDF download

Systemic Polyarteritis Nodosa in the Young: A Single‐Center Experience Over Thirty‐Two Years

Polyarteritis nodosa (PAN) is a rare disease of childhood. The aims of this study were to describe the clinical features, treatment, and outcome of systemic childhood PAN and to identify predictors of relapse. A single-center retrospective medical records review of children with PAN fulfilling the European League Against Rheumatism (EULAR)/Paediatric Rheumatology European Society (PRES)/Paediatric Rheumatology International Trials Organisation (PRINTO) classification criteria who were seen over a 32-year period was performed. Data on demographic and clinical features, treatments, relapses (recurrence of clinical signs/symptoms or occurrence of new symptoms after initial remission requiring escalation or resumption of immunosuppressive therapy), and deaths were recorded. A disease activity score was retrospectively assigned using the Paediatric Vasculitis Activity Score (PVAS) instrument. Cox regression analysis was used to identify significant predictors of relapse. Sixty-nine children with PAN were identified; 55% were male, and their median age was 8.5 years (range 0.9-15.8 years). Their clinical features at presentation were fever (87%), myalgia (83%), skin (88%), renal (19%), severe gastrointestinal (GI) (10%), and neurologic (10%) involvement. The PVAS at presentation was 9 of 63 (range 4-24). Histopathologic analysis of the skin showed necrotizing vasculitis in biopsy samples from 40 of 50 children. Results of selective visceral arteriography suggested the presence of PAN in 96% of patients. Treatment included cyclophosphamide and corticosteroids (83%), plasma exchange (9%), and biologic agents (after 2002; 13%). The relapse rate was 35%, and the mortality rate was 4%. Severe GI involvement was associated with increased risk of relapse (P = 0.031), while longer time to induce remission (P = 0.022) and increased cumulative dose of cyclophosphamide (P = 0.005) were associated with lower relapse risk. Childhood PAN is a severe inflammatory disease of insidious onset and variable clinical presentation. Relapses occurred more frequently in those with severe GI involvement. A higher cumulative dose of cyclophosphamide was associated with a lower risk of relapse.

Impact of the European paediatric legislation in paediatric rheumatology: past, present and future

Conducting clinical trials in paediatric rheumatology has been difficult mainly because of the lack of funding for academic studies and the lack of interest by pharmaceutical companies in the small...

Ultrasound definition of tendon damage in patients with rheumatoid arthritis. Results of a OMERACT consensus-based ultrasound score focussing on the diagnostic reliability

ObjectiveTo develop the first ultrasound scoring system of tendon damage in rheumatoid arthritis (RA) and assess its intraobserver and interobserver reliability.MethodsWe conducted a Delphi study on ultrasound-defined tendon damage and...

Multinational evidence-based recommendations for the diagnosis and management of gout: integrating systematic literature review and expert opinion of a broad panel of rheumatologists in the 3e initiative

We aimed to develop evidence-based multinational recommendations for the diagnosis and management of gout. Using a formal voting process, a panel of 78 international rheumatologists developed 10 key clinical questions...

Advances from clinical trials in juvenile idiopathic arthritis

Treatments available to children with juvenile idiopathic arthritis (JIA) have improved dramatically in the past 15 years, largely because of the development of powerful new biologic treatments. However, the seeds of this development were sewed over 40 years ago with the formation of a group of paediatric rheumatologists who understood the necessity of performing clinical trials in children with JIA. From there, international paediatric rheumatology networks have grown, and are dedicated to and highly experienced in performing such clinical trials. Development of validated outcomes and methodologies has also been critical. The ability to perform these trials stems from legislation enabling the FDA and the European Medicines Agency to require studies to be performed in children before they can be licensed for use in children. Current efforts to enhance the understanding of treatment options for patients with JIA include the development of disease-specific rather than drug-specific consolidated registries, studies in personalized predictive medicine and the development of treatment protocols for regular clinical care of these patients.

FRI0438 Disease management of ankylosing spondylitis and non-radiographic axial spondyloarthritis patients is similar in academic and community practice among 654 surveyed rheumatologists

BackgroundThe Assessment of SpondyloArthritis International Society (ASAS) has recently updated recommendations for the use of anti-TNF in patients (pts) with axial SpondyloArthritis (axSpA), which include ankylosing spondylitis (AS) and non-radiographic...