2013 Update of the 2011 American College of Rheumatology Recommendations for the Treatment of Juvenile Idiopathic Arthritis: Recommendations for the Medical Therapy of Children With Systemic Juvenile Idiopathic Arthritis and Tuberculosis Screening Among Children Receiving Biologic Medications
TL;DR: Guidelines and recommendations developed and/or endorsed by the American College of Rheumatology are intended to provide guidance for particular patterns of practice and not to dictate the care of a particular patient.
Abstract: Guidelines and recommendations developed and/or endorsed by the American College of Rheumatology (ACR) are intended to provide guidance for particular patterns of practice and not to dictate the care of a particular patient. The ACR considers adherence to these guidelines and recommendations to be voluntary, with the ultimate determination regarding their application to be made by the physician in light of each patient's individual circumstances. Guidelines and recommendations are intended to promote beneficial or desirable outcomes but cannot guarantee any specific outcome. Guidelines and recommendations developed or endorsed by the ACR are subject to periodic revision as warranted by the evolution of medical knowledge, technology, and practice.
The American College of Rheumatology is an independent, professional, medical and scientific society which does not guarantee, warrant, or endorse any commercial product or service.
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University of Siena1, Boston Children's Hospital2, University of Florence3, University of Naples Federico II4, University of Brescia5, University of Messina6, University of Palermo7, University of Bari8, Seconda Università degli Studi di Napoli9, University of Padua10, Catholic University of the Sacred Heart11
TL;DR: In this article, the authors investigated the drug retention rate of interleukin (IL)-1 inhibitors on systemic JIA (sJIA) patients and evaluated predictive factors of drug survival based on data from a real-world setting concerning sJIA.
Abstract: Background and Objectives: Few studies have reported the drug retention rate (DRR) of biologic drugs in juvenile idiopathic arthritis (JIA), and none of them has specifically investigated the DRR of interleukin (IL)-1 inhibitors on systemic JIA (sJIA). This study aims to describe IL-1 inhibitors DRR and evaluate predictive factors of drug survival based on data from a real-world setting concerning sJIA. Methods: Medical records from sJIA patients treated with anakinra (ANA) and canakinumab (CAN) were retrospectively analyzed from 15 Italian tertiary referral centers. Results: Seventy seven patients were enrolled for a total of 86 treatment courses. The cumulative retention rate of the IL-1 inhibitors at 12-, 24-, 48-, and 60-months of follow-up was 79.9, 59.5, 53.5, and 53.5%, respectively, without any statistically significant differences between ANA and CAN (p = 0.056), and between patients treated in monotherapy compared to the subgroup co-administered with conventional immunosuppressors (p = 0.058). On the contrary, significant differences were found between biologic-naive patients and those previously treated with biologic drugs (p = 0.038) and when distinguishing according to adverse events (AEs) occurrence (p = 0.04). In regression analysis, patients pre-treated with other biologics (HR = 3.357 [CI: 1.341-8.406], p = 0.01) and those experiencing AEs (HR = 2.970 [CI: 1.186-7.435], p = 0.020) were associated with a higher hazard ratio of IL-1 inhibitors withdrawal. The mean treatment delay was significantly higher among patients discontinuing IL-1 inhibitors (p = 0.0002). Conclusions: Our findings suggest an excellent overall DRR for both ANA and CAN that might be further augmented by paying attention to AEs and employing these agents as first-line biologics in an early disease phase.
615 citations
University of Genoa1, University of Toronto2, Cincinnati Children's Hospital Medical Center3, Utrecht University4, Royal Children's Hospital5, Alfaisal University6, University of Barcelona7, University of Ljubljana8, London Health Sciences Centre9, University of Liverpool10, Hospital General de México11, University of Münster12, Nippon Medical School13, Boston Children's Hospital14, Necker-Enfants Malades Hospital15, Istituto Giannina Gaslini16, Sao Paulo State University17, University of Cape Town18, University of Washington19, Meir Medical Center20, Tel Aviv University21, Medical University of Vienna22
TL;DR: Recommendations for treating JIA to target, being aware that the evidence is not strong and needs to be expanded by future research, can inform various stakeholders about strategies to reach optimal outcomes for JIA.
Abstract: Recent therapeutic advances in juvenile idiopathic arthritis (JIA) have made remission an achievable goal for most patients. Reaching this target leads to improved outcomes. The objective was to develop recommendations for treating JIA to target. A Steering Committee formulated a set of recommendations based on evidence derived from a systematic literature review. These were subsequently discussed, amended and voted on by an international Task Force of 30 paediatric rheumatologists in a consensus-based, Delphi-like procedure. Although the literature review did not reveal trials that compared a treat-to-target approach with another or no strategy, it provided indirect evidence regarding an optimised approach to therapy that facilitated development of recommendations. The group agreed on six overarching principles and eight recommendations. The main treatment target, which should be based on a shared decision with parents/patients, was defined as remission, with the alternative target of low disease activity. The frequency and timeline of follow-up evaluations to ensure achievement and maintenance of the target depend on JIA category and level of disease activity. Additional recommendations emphasise the importance of ensuring adequate growth and development and avoiding long-term systemic glucocorticoid administration to maintain the target. All items were agreed on by more than 80% of the members of the Task Force. A research agenda was formulated. The Task Force developed recommendations for treating JIA to target, being aware that the evidence is not strong and needs to be expanded by future research. These recommendations can inform various stakeholders about strategies to reach optimal outcomes for JIA.
202 citations
University of British Columbia1, University of Manitoba2, Dalhousie University3, University of Saskatchewan4, Centre Hospitalier Universitaire de Sherbrooke5, McGill University Health Centre6, London Health Sciences Centre7, University of Toronto8, Alberta Children's Hospital9, Children's Hospital of Eastern Ontario10, McMaster University11, Janeway Children's Health and Rehabilitation Centre12, Laval University13, Université de Montréal14, University of Alberta15
TL;DR: Most children with JIA managed with contemporary treatments attain inactive disease within 2 years of diagnosis and many are able to discontinue treatment, except for children with polyarthritis.
Abstract: Objective To describe clinical outcomes of juvenile idiopathic arthritis (JIA) in a prospective inception cohort of children managed with contemporary treatments. Methods Children newly diagnosed with JIA at 16 Canadian paediatric rheumatology centres from 2005 to 2010 were included. Kaplan–Meier survival curves for each JIA category were used to estimate probability of ever attaining an active joint count of 0, inactive disease (no active joints, no extraarticular manifestations and a physician global assessment of disease activity 12 months after discontinuing treatment) and of receiving specific treatments. Results In a cohort of 1104 children, the probabilities of attaining an active joint count of 0 exceeded 78% within 2 years in all JIA categories. The probability of attaining inactive disease exceeded 70% within 2 years in all categories, except for RF-positive polyarthritis (48%). The probability of discontinuing treatment at least once was 67% within 5 years. The probability of attaining remission within 5 years was 46–57% across JIA categories except for polyarthritis (0% RF-positive, 14% RF-negative). Initial treatment included joint injections and non-steroidal anti-inflammatory drugs for oligoarthritis, disease-modifying antirheumatic drugs (DMARDs) for polyarthritis and systemic corticosteroids for systemic JIA. Conclusions Most children with JIA managed with contemporary treatments attain inactive disease within 2 years of diagnosis and many are able to discontinue treatment. The probability of attaining remission within 5 years of diagnosis is about 50%, except for children with polyarthritis.
196 citations
Seattle Children's1, Cincinnati Children's Hospital Medical Center2, University of Alabama at Birmingham3, McMaster University4, Children's Mercy Hospital5, National Institutes of Health6, University of Toronto7, Roy J. and Lucille A. Carver College of Medicine8, Virginia Commonwealth University9, University of British Columbia10, Columbia University Medical Center11, Brigham and Women's Hospital12, Medical University of South Carolina13, Duke University14, Boston Children's Hospital15, American College of Rheumatology16, ECRI Institute17
TL;DR: In this paper, the authors developed treatment recommendations for children with juvenile idiopathic arthritis manifesting with non-systemic polyarthritis, sacroiliitis, or enthesitis.
Abstract: Objective:
To develop treatment recommendations for children with juvenile idiopathic arthritis manifesting with non-systemic polyarthritis, sacroiliitis, or enthesitis.
193 citations
TL;DR: This review highlights the pathogenesis of MAS/sHLH including its underlying triggers, key clinical features and diagnostic challenges, prognostic factors and current treatments in adults.
Abstract: Haemophagocytic lymphohistiocytosis (HLH) is a hyperinflammatory syndrome, which if not promptly treated, can lead rapidly to critical illness and death. HLH is termed macrophage activation syndrome (MAS) when associated with rheumatic disease (where it is best characterized in systemic JIA) and secondary HLH (sHLH) when associated with other triggers including malignancy and infection. MAS/sHLH is rare and coupled with its mimicry of other conditions, is underrecognized. These inherent challenges can lead to diagnostic and management challenges in multiple medical specialties including haematology, infectious diseases, critical care and rheumatology. In this review we highlight the pathogenesis of MAS/sHLH including its underlying triggers, key clinical features and diagnostic challenges, prognostic factors and current treatments in adults.
145 citations
References
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Journal Article•
University of British Columbia1, University of Birmingham2, University of Western Australia3, University of Rochester4, Cincinnati Children's Hospital Medical Center5, Federal University of São Paulo6, Capital Medical University7, University of Buenos Aires8, University of Guadalajara9, University of Paris10, University College London11
TL;DR: The second revision of the ILAR Taskforce on Classification of Childhood Arthritis (ILAR-JIA) was presented at the 2001 ILAR Workshop on Rheumatology as discussed by the authors.
Abstract: The primary aim of the International League of Associations for Rheumatology (ILAR) proposals for classification of juvenile idiopathic arthritis (JIA) is to delineate, for research purposes, relatively homogeneous, mutually exclusive categories of idiopathic childhood arthritis based on predominant clinical and laboratory features. As part of a continuing review process, the ILAR Taskforce on Classification of Childhood Arthritis met in Edmonton in 2001 to discuss modifications to the proposed JIA classification. Since the publication of the first revision of the original classification 1 , a number of descriptive studies using the new classification have been reported 2-11. The aims of this communication are 2-fold: to outline modifications to the revised classification proposed as a result of the Edmonton meeting, and to correct misconceptions highlighted by the published studies concerning the clinical use of the classification. The Edmonton Revision The changes embodied in the second revision of the classification are as follows: 1. Clarification of the definitions of each category. 2. Improvement in the congruity between inclusion and exclusion criteria. 3. Removal of the requirement that a dermatologist make the diagnosis of psoriasis. 4. Removal of the requirement that there be medical confirmation of HLA-B27 associated disease in a relative. 5. Reduction in the age for criterion " 3 " of enthesitis related arthritis, and exclusion " b " from 8 years to 6 years of age. 6. Improvement in the consistency of the structure. The impracticality of the requirement that a diagnosis of psoriasis be made by a dermatologist was recognized, and this requirement was modified so that the diagnosis of psori-asis could be made by a physician (not necessarily a dermatologist). Similarly, it is no longer required that there be medical confirmation of an HLA-B27 associated disease in a relative as contained in exclusion " c. " It is evident that it is very difficult to obtain a reliable history of psoriasis or an HLA-B27 associated disease in a second-degree relative. Therefore, a history of importance to the application of the criteria is restricted to the patient or a first-degree relative (parents or siblings) only. The study of Murray, et al 8 indicated that the HLA-B27 association is important in boys over the age of 6 years at onset of arthritis, and this age was substituted for 8 years in exclusion " b. " Discrepancies between inclusion and exclusion criteria were resolved, and the exclusions were identified by the letters …
3,201 citations
01 Jan 2004
TL;DR: The aims of this communication are to outline modifications to the revised classification proposed as a result of the Edmonton meeting, and to correct misconceptions highlighted by the published studies concerning the clinical use of the classification.
2,851 citations
TL;DR: The Report of the Committee on Infectious Disease (ie, the so-called Red Book) is a source of essential information on the prevention, diagnosis, and treatment of pediatric infectious diseases.
Abstract: Infectious diseases are the most common pediatric problems facing primary care practitioners and are a frequent concern for dermatologists. Although in North America most of these infections are not serious, their prevention and treatment require familiarity with a myriad of facts, including immunization schedules, incubation periods, diagnostic tests, and treatment regimens. To make matters worse, there are frequent changes in accepted treatment and prevention regimens. The retention of all of this information is probably impossible and certainly not necessary. The Report of the Committee on Infectious Disease (ie, the so-called Red Book) is a source of essential information on the prevention, diagnosis, and treatment of pediatric infectious diseases. The information is easily retrievable through an excellent table of contents and index. It is compiled by the Committee on Infectious Diseases of the American Academy of Pediatrics. It is authoritative and up to date with a new edition published every 2
2,064 citations
01 Jan 2001
TL;DR: The "RAND/UCLA Appropriateness Method" was developed by RAND and UCLA in the 198Os and has been further developed and refined in North America and, increasingly, in Europe.
Abstract: : The concepts of appropriate and necessary care are fundamental to the creation of an efficient and equitable health-care delivery system Evidence of inappropriate overuse and underuse of procedures has been documented even in health systems characterised by the absence of global budgets, capitation, utilisation review or the pressure of requiring a second opinion Health systems should function in such a way that inappropriate care is progressively reduced, while appropriate and especially necessary care are maintained or increased The ability to determine and identify which care is overused and which is underused is essential to this functioning To this end, the "RAND/UCLA Appropriateness Method" (here given the acronym RAM) was developed by RAND and UCLA in the 198Os It has been further developed and refined in North America and, increasingly, in Europe
1,855 citations
25 Jun 2010
TL;DR: In this paper, the authors provide guidance to U.S. public health officials, health care providers, and laboratory workers for use of FDA-approved IGRAs in the diagnosis of M. tuberculosis infection in adults and children.
Abstract: n 2005, CDC published guidelines for using the QuantiFERON-TB Gold test (QFT-G) (Cellestis Limited, Carnegie, Victoria, Australia) (CDC. Guidelines for using the QuantiFERON-TB Gold test for detecting Mycobacterium tuberculosis infection, United States. MMWR;54[No. RR-15]:49-55). Subsequently, two new interferon gamma (IFN- gamma) release assays (IGRAs) were approved by the Food and Drug Administration (FDA) as aids in diagnosing M. tuberculosis infection, both latent infection and infection manifesting as active tuberculosis. These tests are the QuantiFERON-TB Gold In-Tube test (QFT-GIT) (Cellestis Limited, Carnegie, Victoria, Australia) and the T-SPOT.TB test (T-Spot) (Oxford Immunotec Limited, Abingdon, United Kingdom). The antigens, methods, and interpretation criteria for these assays differ from those for IGRAs approved previously by FDA. For assistance in developing recommendations related to IGRA use, CDC convened a group of experts to review the scientific evidence and provide opinions regarding use of IGRAs. Data submitted to FDA, published reports, and expert opinion related to IGRAs were used in preparing these guidelines. Results of studies examining sensitivity, specificity, and agreement for IGRAs and TST vary with respect to which test is better. Although data on the accuracy of IGRAs and their ability to predict subsequent active tuberculosis are limited, to date, no major deficiencies have been reported in studies involving various populations. This report provides guidance to U.S. public health officials, health-care providers, and laboratory workers for use of FDA-approved IGRAs in the diagnosis of M. tuberculosis infection in adults and children. In brief, TSTs and IGRAs (QFT-G, QFT-GIT, and T-Spot) may be used as aids in diagnosing M. tuberculosis infection. They may be used for surveillance purposes and to identify persons likely to benefit from treatment. Multiple additional recommendations are provided that address quality control, test selection, and medical management after testing. Although substantial progress has been made in documenting the utility of IGRAs, additional research is needed that focuses on the value and limitations of IGRAs in situations of importance to medical care or tuberculosis control. Specific areas needing additional research are listed.
1,074 citations