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Children with suspected localized scleroderma should see specialist
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All children suspected of having localized scleroderma should be referred to a specialized pediatric rheumatology center, according to recommendations published in the Annals of the Rheumatic Diseases.
“In 2012, a European project called Single Hub and Access point for pediatric Rheumatology in Europe (SHARE) was launched to optimize and disseminate diagnostic and management regimens in Europe for children and young adults with rheumatic diseases,” Francesco Zulian, MD, of the University of Padova, Italy, and colleagues wrote. “As currently no international or European consensus exists with regard to the assessment and treatment of juvenile rheumatic diseases, defining clear guidelines is one of the most important aims of the SHARE initiative.”
Focusing on juvenile localized scleroderma, Zulian and colleagues aimed to establish assessment and treatment recommendations for the disease, to be devised by a 15-member committee of experts in pediatric rheumatology. The committee included 10 members of the SHARE consortium, with the remaining five recruited for their clinical experience in juvenile localized scleroderma. First, members of the committee conducted a systematic literature search of studies on the diagnosis, assessment and treatment of juvenile localized scleroderma, initially netting 1,550 papers. In all, 53 studies were selected for consideration.
Information from the selected studies was then validated and examined by two experts on the committee, who used the data to draft 18 provisional recommendations. These draft recommendations were presented to the committee via online survey and later revised. They were then presented to the full committee for discussion during two face-to-face meetings in March 2014, in Italy, and March 2015, in Spain.
The committee ultimately accepted one overarching principle and 16 recommendations — 10 on assessment and six covering treatment — all with at least 80% agreement. Topics include the assessment of skin and extracutaneous involvement, as well as suggested treatments. The overarching principle, accepted with 100% agreement, was “all children with suspected localized scleroderma should be referred to a specialized pediatric rheumatology center.”
Recommendations for assessment urged that specialized ultrasound imaging, as well as standardized assessment and color Doppler, could be useful for determining disease activity and treatment response. In addition, all children with juvenile localized scleroderma should be evaluated, at diagnosis and during follow-up, with a complete joint examination, including the temporomandibular joint.
All six treatment recommendations were approved by the committee with 100% agreement. They suggest that:
- Systemic corticosteroids could be useful in the disease’s inflammatory phase, and that methotrexate or another disease-modifying antirheumatic drug should be started in combination with corticosteroids;
- All patients at risk for disfigurement or disability in relation to the disease should receive 15 mg/m2 of oral or subcutaneous methotrexate per week;
- Methotrexate should be maintained for at least 12 months before tapering in the event of acceptable clinical improvement;
- Mycophenolate mofetil can be used for severe forms of the disease in patients who are unresponsive of, or intolerant to, methotrexate;
- Ultraviolet A1 phototherapy in medium doses can be used to improve skin softness in isolated morphea lesions; and
- Skin thickening of circumscribed morphea can be treated using topical imiquimod.
“This SHARE initiative is based on expert opinion informed by the best available evidence and provides recommendations for the diagnosis and treatment of patients with [juvenile localized scleroderma], along with other pediatric rheumatic diseases, with a view to improving their outcome in Europe,” Zulian and colleagues wrote. “We anticipate that these guidelines will likely be adopted by physicians caring for patients with [juvenile localized scleroderma] outside Europe. It will now be important to broaden discussion and test the reliability of these recommendations to the wider scientific community and to the patients.” – by Jason Laday
Disclosure: Zulian reports no relevant financial disclosures. Please see the study for all other relevant financial disclosures.
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