Rheumatologic Conditions

Children & Adolescents

Lead Author(s): 

Aimee O. Hersh, MD

Supporting Author(s): 

Sylvia I. Watkins-Castillo, PhD

An estimated 300,000 children in the Unites States are diagnosed with juvenile arthritis or another chronic rheumatologic condition such as systemic lupus erythematosus, juvenile dermatomyositis, or linear scleroderma.1 These chronic musculoskeletal conditions generally require chronic care, and without appropriate treatment can lead to significant disability.

Juvenile idiopathic arthritis (JIA) (formally called juvenile rheumatoid arthritis [JRA] or juvenile chronic arthritis [JCA]) is estimated to affect 1 in 1,000 children in the United States.2 JIA is diagnosed in a child younger than 16 years of age with at least six weeks of persistent arthritis. There are seven distinct subtypes, each having a different presentation and association to autoimmunity and genetics.3 Certain subtypes are associated with an increased risk of inflammatory eye disease (uveitis). Understanding the differences in the various forms of JIA, their causes, and methods to better diagnose and treat these conditions in children is important to future treatment and prevention. Among all subtypes, approximately half of children with JIA still have active disease after 10 years.4

There are several other causes of acute or chronic arthritis in children that do not meet the diagnostic criteria of JIA, including, but not limited to, rheumatic fever, Reiter syndrome/reactive arthritis, and the arthritis associated with inflammatory bowel disease.

Approximately 15% to 20% of cases of systemic lupus erythematosus (SLE) in the United States occur in children younger than 18 years of age. SLE is a chronic autoimmune condition characterized by the production of autoantibodies leading to immune complex formation and end organ damage. For reasons that remain unclear, pediatric SLE is associated with increased disease severity, increased short- and long-term morbidity, and mortality as compared to adult-onset SLE.5

Juvenile dermatomyositis is a chronic inflammatory condition characterized by inflammation of the skin and muscle. Estimated incidence of the disease in the United States is 0.5 per 100,000 people; the prevalence is not known.2

The sclerodermatous conditions are defined in part by the common clinical feature of tightening or hardening of the skin. Systemic scleroderma, also called diffuse cutaneous systemic scleroderma, is rare in childhood, accounting for only 2% to 3% of all cases of this condition, which has an estimated prevalence of 24 cases per 100,000 people. Linear scleroderma is the most common subtype of scleroderma diagnosed in the pediatric population. It is characterized by a linear streak of sclerosis typically involving an upper or lower extremity.2

In 2006, the CDC Arthritis Program finalized a case definition for ongoing surveillance of pediatric arthritis and other rheumatologic conditions (SPARC) using the current ICD-9-CM diagnostically -based data systems.6 In response to the variations in conditions that some felt should be included but were not, CDC generated estimates not included in the case definition.

  • 1. Sacks JJ, Helmick CG, Luo YH, Ilowite NT, Bowyer S: Prevalence of and annual ambulatory health care visits for pediatric arthritis and other rheumatologic conditions in the United States in 2001–2004. Arthritis Rheum 2007;57(8):1439-1445.
  • 2. a. b. c. Cassidy JT, Petty RE, Laxer RM, Lindsley CB: Textbook of Pediatric Rheumatology, 6th ed. 2010. Elsevier Inc, Philadelphia, PA.
  • 3. Petty RE, Southwood TR, Manners P, et al: International League of Associations for Rheumatology classification of juvenile idiopathic arthritis: Second revision, Edmonton, 2001. J Rheumatol 2004;31(2):390-392.
  • 4. Minden, K: Adult outcomes of patients with juvenile idiopathic arthritis. Horm Res 2009;72(Suppl 1)20-25.
  • 5. Kamphuis S, Silverman ED: Prevalence and burden of pediatric-onset systemic lupus erythematosus. Nat Rev Rheumatol 2010;6(9):538-546.
  • 6. Centers for Disease Control and Prevention (CDC): Childhood arthritis. Available at: (http://www.cdc.gov/arthritis/basics/childhood.htm) Accessed February 19, 2015.


  • 2014

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