LONDON – Recommendations on managing juvenile idiopathic arthritis and connective tissue disorders in children and young people across Europe were unveiled at the European Congress of Rheumatology.
The recommendations, which come from the SHARE (Single Hub and Access Point for Paediatric Rheumatology in Europe) project, cover best practices and provide guidance based on current evidence and expert opinion for the optimal diagnosis and treatment of these rare rheumatic diseases that affect the pediatric population.
It is hoped that the recommendations will be used to improve access to treatment and care within individual countries such that a child in one country will be able to receive the same standard of care as a child in another, Dr. Nico Wulffraat of University Medical Center Utrecht (the Netherlands) said in an interview.
Dr. Wulffraat, one of the driving forces behind the project, noted that the SHARE project was set up to look at making the management of rare pediatric rheumatic diseases more uniform across Europe. It addressed conditions such as juvenile idiopathic arthritis (JIA), childhood-onset systemic lupus erythematosus (cSLE), childhood antiphospholipid syndrome (APS), childhood vasculitis, juvenile dermatomyositis, and pediatric scleroderma. In addition, recommendations on diagnosis and treatment of periodic fever syndromes have been developed in collaboration with experts from the Eurofever Project.
“Our evidence- and consensus-based recommendations will hopefully drive access to uniform and optimal care throughout Europe, including off-label therapy when appropriate according to international consensus–derived expert advice,” Dr. Sebastiaan Vastert, SHARE project co-coordinator, said in an interview. He added: “The SHARE network will be invaluable for further international collaboration, both for optimization of care and for international collaboration in research as well.”
Dr. Wulffraat observed that while the recommendations are primarily directed at health care professionals, they also are of use for other stakeholders such as health authorities and insurance companies, and of course patients themselves to ensure the best level of care is being achieved throughout Europe.
The process for developing the guidelines was perhaps as important as the recommendations themselves, said Dr. Vastert, also of University Medical Center Utrecht. The process helped to build a network of international experts who could work together to develop future recommendations for improving patient care.
The recommendations for JIA and other pediatric rheumatic diseases included 51 “cross-cutting” statements, Dr. Vastert said. One of these statements was that a pediatric rheumatologist should manage children with signs of rheumatic disease. Another highlighted the members of a multidisciplinary team who should be involved as appropriate, such as a nurse specializing in pediatric rheumatic disease, a physiotherapist or occupational therapist, and a psychologist or psychosocial worker. Dr. Vastert also noted that good communication between team members is essential. In addition, there needs to be clear guidance on when to refer to a pediatric rheumatologist.
The SHARE project JIA recommendations include 10 evidence-based statements on diagnosis, 31 evidence-based statements on treatment, and 17 general statements on specific care for JIA, Dr. Vastert said. A few examples of the latter are that new patients should be seen in a specialist center within 4 weeks of referral; new patients and those starting a new therapy should be reviewed within 2-3 months to check on adherence, tolerance, and disease progression; and monitoring response to ongoing treatment should be every 3-6 months, preferably using existing standardized disease activity tools.
EULAR standard operating procedures were followed when developing the various SHARE recommendations, said Dr. Michael Beresford of the University of Liverpool (England) and the lead for the recommendations on childhood connective tissue disorders. Dr. Beresford noted that the latter were a rare, and in some cases extremely rare, complex group of pediatric rheumatic diseases that could lead to significant morbidity and mortality.
“Evidence-based guidelines have been lacking, and management is based mainly on physician experience. Consequently, treatment regimens vary widely throughout Europe,” Dr. Beresford observed. “These [recommendations] provide evidence-based, internationally agreed-upon standards of optimal care for pediatric connective tissue disorders.”
Specifically, the connective tissue disorder recommendations cover when to refer and how to diagnose, treat, and monitor cSLE (including neuropsychiatric SLE), childhood APS, and juvenile vasculitides, including rare pediatric vasculitides such as Takayasu arteritis. The SHARE recommendations for the management of juvenile dermatomyositis are currently in press in Annals of the Rheumatic Diseases, Dr. Beresford said.
Giving a few examples of recommendations for cSLE, Dr. Beresford noted that one of the challenges is to try to prevent delay in diagnosis. The expert panel decided that the 2012 Systemic Lupus International Collaborating Clinics (SLICC) criteria could be used for diagnosis. A referral to a pediatric rheumatologist is warranted, they determined, when a child has a positive antinuclear antibody (ANA) test and meets two clinical SLICC criteria. Dr. Beresford conceded that antibody testing might not be available because of cost in all countries, but they “decided to draw a line in the sand” to say that it is important that it is routinely done in order to come closer to a definitive diagnosis.