Juvenile systemic sclerosis is a very rare orphan disease. To date, only one publication has estimated the prevalence of juvenile systemic sclerosis using a survey of specialized physicians. We conducted a study of administrative claims data in the United States using the International Classification of Diseases, Ninth Revision diagnosis codes and found a prevalence of approximately 3 per 1,000,000 children. This estimate will inform the planning of prospective studies.
MartiniGFoeldvariIRussoRet al. Systemic sclerosis in childhood: clinical and immunologic features of 153 patients in an international database. Arthritis Rheum2006; 54(12): 3971–3978.
2.
FoeldvariIZhavaniaMBirdiNet al. Favourable outcome in 135 children with juvenile systemic sclerosis: results of a multi-national survey. Rheumatology2000; 39(5): 556–559.
3.
HerrickALEnnisHBhushanMet al. Incidence of childhood linear scleroderma and systemic sclerosis in the UK and Ireland. Arthritis Care Res2010; 62(2): 213–218.
