Rheumatoid Arthritis, Systemic Juvenile

Alternative Names

  • Systemic Juvenile Rheumatoid Arthritis
  • Juvenile Rheumatoid Arthritis
  • JRA
  • Juvenile Chronic Arthritis
  • JCA
  • Juvenile Idiopathic Arthritis
  • JIA
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WHO-ICD-10 version:2010

Diseases of the musculoskeletal system and connective tissue

Arthropathies

OMIM Number

604302

Description

Juvenile rheumatoid arthritis (JRA) is one of the most common chronic diseases of childhood, and is characterized by chronic joint inflammation. Three major subtypes of the condition are recognized based on the extent of joint involvement; pauciarticular/oligoarticular onset type (with four or lesser number of joints involved), polyarticular type (with more than four joints involved), and systemic onset type (with fever, rash, and systemic arthritis).

The condition manifests itself between the ages of 6-months and 16-years. Initial symptoms of the condition include joint pain, swelling, reddened, or warm joints. Affected children usually have generalized growth abnormality that can be abrogated by growth hormone supplementation. Clinically, the child may present with a limp and joint swellings. X-ray may reveal soft tissue swelling.

Treatment of JRA involves a combination of medication, physical therapy, and exercise. Inflammation and pain can be managed with the use of Non-Steroidal Anti Inflammatory Drugs (NSAIDs), and other medications. A regular exercise program is very important, as strengthening the muscles is a vital part of providing support and protection to the joints.

The condition is rarely fatal. Only in 4% of the systemic cases, do affected children die of infection and amyloidosis. In most of those affected with the pauciarticular type, the condition resolves itself, and children regain normal function. In about 15% of these children, however, the condition may worsen, while, 10% may have eye diseases progressing to blindness. Children affected with the systemic form, also, for the most part, tend to have a resolution of their clinical features. The worst prognosis is for those whose disease has onset before 5-years of age. A worldwide prevalence of 1-2 per 1,000 children has been estimated for JRA. Females show a more pronounced frequency for the pauciarticular and polyarticular types.

Molecular Genetics

The etiology of JRA is not clearly known, although several predisposing factors have been identified. Several researchers have considered a possible infectious etiology, especially a viral origin. However, no virus has yet been isolated from affected children.

Susceptibility to JRA has been attributed to HLA Class Molecules. HLA DR1 and DR4 have been shown to be susceptible to the sero-positive polyarticular type, while the polyarticular JRA is associated with HLA DR5, DR8, DQW1, and DPW2. Systemic onset JRA has been shown to be associated with HLA DR4.

Epidemiology in the Arab World

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Other Reports

Kuwait

Khuffash et al. (1990) conducted a hospital-based retrospective study over a period of 8-years on the epidemiology of arthritis and other connective tissue disorders among children in Kuwait. A total of 108 children under the age of 12-years with JCA were identified, giving an average annual incidence of 2.84/100,000. This was lower than the 1988 prevalence of 18.7/100,000. Among the subtypes, polyarticular type was the most common (42%), followed by the systemic, and oligoarticular types (29% each).

Al Saeid and Al Onaizi (1997) presented an epidemiologic survey of 47 children diagnosed with juvenile rheumatoid arthritis (JRA). The findings of this study suggested a prevalence of 0.36 cases per 1,00 children at risk. Upon investigating JRA subtypes, Al Saeid and Al Onaizi (1997) found that the predominant subtype; pauciarticular onset accounted for 49% while polyarticular and systemic onset type accounted for 25.5% each. On the other hand Iridocyclitis was diagnosed in 3 children [6.4%] only and is therefore, infrequent in Kuwaitis while other epidemiological parameters were comparable to international figures. Six years later, Alsaeid et al. (2003) investigated the incidence of angiotensin converting enzyme (ACE) gene insertion-deletion (I/D) polymorphism genotypes in 82 children with juvenile rheumatoid arthritis (JRA) and in 48 ethnically matched healthy controls. A considerably higher incidence of II genotype was observed in the JRA patients compared to controls (p < 0.003). In contrast, no statistically significant difference was detected in the incidence of DD and ID genotypes in JRA patients and controls (p = 0.276 and 0.460, respectively). By analyzing the incidence of ACE gene polymorphism genotypes with regard to the clinical subclasses of JRA, the incidence of II genotype was found to be significantly higher in all the 3 JRA subclasses compared to controls. The strongest association between II genotype and JRA subclasses was detected in systemic JRA, followed by oligoarticular and polyarticular JRA. This was also reflected in a higher prevalence of I-allele in the systemic JRA cases (13/26, 50%) compared to the D-allele (11/26, 42%).

[Al Saeid K, Al Onaizi E. Pattern of juvenile rheumatoid arthritis in a teaching hospital in Kuwait. Med Princ Pract. 1997; 6(1):22-5.]

 

Saudi Arabia

Al-Mayouf (2007) undertook a retrospective review of children who presented with arthropathy and a positive family history of a similar condition between 1990 and 2005 in a tertiary hospital in Saudi Arabia. Of the 62 patients thus identified, 12 (9 females, 3 males) were diagnosed with Familial Idiopathic Juvenile Arthrits (FJIA). All 12 patients came from the same region, and presented with multiple joint involvements. Of the 12, four had a systemic onset subtype. They were treated with NSAIDs, systemic corticosteroids, and antirheumatic drugs. The presence of parental consanguinity and a positive family history along with multiple affected siblings supported the hereditary nature of the disease, and Al-Mayouf (2007) suggested an autosomal recessive mode of inheritance.

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