Rheumatology

Childhood Scleroderma

This study occurred from July 2005 to July 2007. At that time, Scleroderma affected children as well as adults, and was associated with significant morbidity and mortality. This study aimed to ascertain the incidence of childhood scleroderma in the UK and Ireland and describe the usual presenting symptoms. It aimed to describe the delay between symptom onset and diagnosis as well as the pattern of care received by affected children before and after diagnosis. Additionally, it aimed to ascertain the demographics of the children affected and describe regional and ethnic variations in incidence

By bpsu · July 1, 2005

Lead investigator

Dr A Herrick

About the study

This study occurred from July 2005 to July 2007. At that time, Scleroderma affected children as well as adults, and was associated with significant morbidity and mortality. While some children with scleroderma had systemic sclerosis (SSc), more commonly, scleroderma in children was localised and confined to the skin and underlying tissues. Two international studies documented the clinical and immunological features of systemic sclerosis and of localised scleroderma in children.

Systemic sclerosis was rare in children, but it was an important condition because internal organ involvement could be life-threatening.

Although localised scleroderma was not a multisystem connective tissue disease, it could be severely debilitating. There was little known about the epidemiology of localised scleroderma in children. The early lesions of localised scleroderma were inflammatory; however, a study had reported extracutaneous features in a significant proportion of children.

Therefore, there was a rationale for early diagnosis and intervention.

This study aimed to:

  • Ascertain the incidence of childhood scleroderma in the UK and Ireland.
  • Describe the usual presenting symptoms.
  • Establish the delay between symptom onset and diagnosis.
  • Determine the pattern of care received by affected children before and after diagnosis.
  • Ascertain the demographics of the children affected.
  • Describe regional and ethnic variations in incidence.

Duration

July 2005 – July 2007

Published papers

Herrick AL, Ennis H, Bhushan M, Silman AJ, Baildam EM. Incidence of childhood linear scleroderma and systemic sclerosis in the UK and Ireland. Arthritis Care Res (Hoboken). 2010 Feb;62(2):213-8. doi: 10.1002/acr.20070. PMID: 20191520.

BPSU 22nd Annual report 2007 -2008

Scleroderma and Raynauds UK
Web: https://www.sruk.co.uk Email; info@sruk.co.uk