Juvenile arthritis (JA) is an umbrella term used to describe a number of autoimmune and inflammatory conditions that can develop in children. It is the most common rheumatic disease of childhood, particularly in the Western world, with children of European descent reporting higher incidence rates.1,2
The most common form of JA is Juvenile Idiopathic Arthritis (JIA) (formally called juvenile rheumatoid arthritis (JRA) or Juvenile Chronic Arthritis (JCA)). JIA is diagnosed in a child <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 Subtypes also differ in typical age of onset. 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, 40% to 45% of children with JIA still have active disease after 10 years.4
Prevalence Of Juvenile Arthritis
Due to the various forms of JA, estimates of prevalence and incidence are difficult to ascertain. Overall estimates are that 294,000 children in the United States have arthritis or another rheumatic disease.5
In 2006, the CDC Arthritis Program finalized a case definition for ongoing surveillance of significant pediatric arthritis and other rheumatologic conditions (SPARC) using the current ICD-9-CM diagnostically based data systems. In response to the variations in conditions that some felt should be included, but were not, CDC generated estimates for conditions that were not included in the case definition but were felt by some should have been.
Healthcare Utilization
Hospitalization for JA
Using the SPARC definitions, analysis of the recent national healthcare database focusing on children, the Healthcare Cost and Utility Project (HCUP) KID, showed 104,400 children age 17 and younger were discharged from a hospital with any diagnosis of SPARC in 2012. Of those, 15,600, or 15%, had an admitting diagnosis of SPARC. Slightly more females than males were hospitalized; children age 6 and younger were more likely to be hospitalized with an admitting diagnosis of SPARC than older children, accounting for 40% of admissions.
Only a small number of children (3.8%) discharged with any diagnosis of SPARC had a diagnosis of juvenile arthritis. Females accounted for 72% of discharges with a diagnosis of JIA, with 62% of the discharges for children age 13 to 17 years.
Average hospital stays of eight days were found for any diagnosis of SPARC. The very youngest children, babies under age one, had much longer stays and higher mean hospital charges. Children with a diagnosis of JIA had hospital stays of a mean of 4.6 days, with subsequently lower mean charges.
Total hospital charges associated with any diagnoses of SPARC in the population younger than age 20 were $8.3 billion in 2012. (Reference Table 3B.1 PDF CSV)
JA Ambulatory Care Visits
Emergency rooms saw 515,600 patients ages 0 to 17 with any diagnoses of SPARC in 2013. Among these patients, 6,900 had a primary diagnosis of JIA. Visits did not show major differences by sex or age.
Due to smaller sample sizes in the currently available databases for physician office visits and outpatient clinics, outpatient visits for a diagnosis of SPARC in the juvenile population are difficult to quantify. In 2013, physician visits for treatment of JA numbered 1.2 million. As with ED visits, major differences by sex or age were not seen. Due to small sample sizes, the number of visits with a diagnosis of JIA was unreliable.
Outpatient clinics saw 305,100 patients in 2011, the most recent year for outpatient data available. Patterns for distribution reflected that of other treatment sites. Due to small sample sizes, the number of visits with a diagnosis of JIA was unreliable.
From these data, an estimated 2.03 million outpatient visits for any diagnoses of SPARC occurred in the 0 to 17 years age population in 2013. (Reference Table 3B.1 PDF CSV)
ICD-9-CM Codes for SPARC
In 2006, the CDC Arthritis Program finalized a case definition for ongoing surveillance of significant pediatric arthritis and other rheumatologic conditions (SPARC) using the current ICD-9-CM diagnostically-based data systems.
099.3 - Reactive arthritis
136.1 - Behcet's syndrome
274 - Gout
277.3 - Amyloidosis (includes Familial Mediterranean Fever)
287.0 - Allergic purpura / Henoch Schonlein purpura
390 - Rheumatic fever without heart involvement
391 - Rheumatic fever with heart involvement
437.4 - Cerebral arteritis
443.0 - Raynaud's syndrome
446 - Polyarteritis nodosa and allied conditions
447.6 - Arteritis, unspecified
695.2 - Erythema nodosum
696.0 - Psoriatic arthropathy
701.0 - Linear scleroderma / Circumscribed scleroderma / Morphea
710 - Diffuse diseases of connective tissue
711 - Arthropathy associated with infections
712 - Crystal arthropathies
713 - Arthropathy associated with other disorders classified elsewhere
714 - Rheumatoid arthritis and other inflammatory polyarthropathies
715 - Osteoarthritis and allied disorders
716 - Other and unspecified arthropathies
719.2 - Villonodular synovitis
719.3 - Palindromic rheumatism
720 - Ankylosing spondylitis and other inflammatory spondylopathies
727.0 - Tenosynovitis
729.0 - Rheumatism, unspecified and fibrositis
729.1 - Myalgia and myositis, unspecified
- 1. Saurenmann RK, Rose JB ,Tyrrell P, et. al. Epidemiology of juvenile idiopathic arthritis in a multiethnic cohort. Arthritis & Rheumatism. 2007;56(6):1974–1984. DOI 10.1002/art.22709.
- 2. Espinosa M, Gottlieb BS. Juvenile Idiopathic Arthritis. Pediatrics in Review 2012;33:303-13.
- 3. Petty RE, Southwood TR, Manners P, et al. for the International League of Associations for Rheumatology. International League of Associations for Rheumatology classification of juvenile idiopathic arthritis: second revision, Edmonton, 2001. J Rheumatol. 2004 Feb;31(2):390-2.
- 4. Arthritis Foundation (http://www.arthritis.org/arthritis-facts/disease-center/juvenile-arthrit...) November 11, 2014.
- 5. Centers for Disease Control and Prevention (CDC). What is juvenile arthritis? (https://www.niams.nih.gov/Health_Info/Juv_Arthritis/juvenile_arthritis_f... ) Accessed March 22, 2017.
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