The British Society for Rheumatology has issued consensus-based recommendations for the diagnosis and treatment of juvenile systemic sclerosis (SSc). These guidelines have been published in Rheumatology.

Juvenile SSc is a rare disease and there are currently no specific guidelines for the diagnosis and management of these patients. In 2012, the Single Hub and Access Point for Pediatric Rheumatology in Europe (SHARE) was established to develop consensus-based recommendations for children and young adults with the rheumatic disease.

For the current consensus-based recommendations for juvenile SSc, a workgroup was established that included an international committee of 16 experts in pediatric rheumatology. Members completed a literature search and assessed the validity and level of evidence of relevant studies to provide drafted recommendations. Overall, the panel formulated 14 recommendations for the management of juvenile SSc.

Recommendations for the Assessment of Juvenile SSc

  • As an overarching principle, the committee agreed that all children with suspected juvenile SSc should be referred to a specialized pediatric rheumatology center for multidisciplinary care.
  • All patients with isolated Raynaud phenomenon should receive nailfold capillary assessment and antinuclear antibody testing, with regular follow-up.
  • The committee recommended that a standardized skin score tool be used for the clinical assessment of patients with juvenile SSc; the modified Rodnan skin score may also be used.
  • All panelists agreed on a complete assessment for internal organ involvement, including pulmonary function testing with diffusing capacity for carbon monoxide and high-resolution computed tomography to determine the presence and severity of interstitial lung disease.
  • Pulmonary function tests with diffusing capacity for carbon monoxide, echocardiography, renal function, and modified Rodnan skin score have been recommended at least every 6 months.
  • The committee noted that the Juvenile SSc Severity Score can be used to assess the severity of juvenile SSc; however, further studies are required to validate its use.

Recommendations for the Treatment of Juvenile SSc

  • Systemic corticosteroids combined with a disease modifying antirheumatic drug have been recommended as the preferred treatment strategy in the active inflammatory phase of juvenile SSc.
  • The panel suggested that systemic immunomodulatory treatment, such as methotrexate, be considered at the time of juvenile SSc diagnosis.
  • In case of treatment failure with methotrexate, an additional immunomodulatory agent may be considered.
  • For patients with cardiac or pulmonary involvement, cyclophosphamide may be used.
  • The committee noted that iloprost may be used in the presence of vascular involvement, such as ischemic digits and digital ulcerations.
  • They added that bosentan be considered for digital ulcerations that are refractory to calcium channel blockers and prostanoids or in case of New York stage 2 pulmonary hypertension.
  • In severe or refractory cases, biologics, including tocilizumab and rituximab, should be considered.
  • The panel suggested the use of autologous stem cell transplantation to treat patients with juvenile SSc with progressive disease refractory to immunosuppressive therapy.

“It is now important to disseminate these recommendations, test their reliability in the international scientific community, and update them as new data become available,” the committee noted.


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Reference

Foeldvari I, Culpo R, Sperotto F, et al. Consensus-based recommendations for the management of juvenile systemic sclerosis. Rheumatology (Oxford). Published online November 4, 2020. doi:10.1093/rheumatology/keaa584