Rheumatoid factor-negative juvenile idiopathic arthritis

Other Names: Rheumatoid factor negative erosive chronic polyarthritis; Rheumatoid factor-negative polyarthritis; RF-ve CP; Juvenile rheumatoid factor-negative polyarthritis; Rheumatoid factor-negative JIA; Polyarthritis without rheumatoid factor

Rheumatoid factor-negative polyarthritis is a term used to describe a group of poorly defined heterogenous conditions that incorporates forms of rheumatoid factor-negative polyarthritis and forms of oligoarticular arthritis that become extensive in less than 6 months after onset.

Epidemiology

Rheumatoid factor-negative polyarthritis represents around 15-20% of cases of juvenile idiopathic arthritis (JIA). Prevalence has been estimated at between 1 and 13 in 22,200 children, with an annual incidence of 1-26 in 700,000 children. Females are affected more often than males.

Cause

The aetiology is difficult to determine as a result of the heterogeneity.

Signs and symptoms

The joint manifestations are bilateral, nearly symmetrical and affect both the large and small joints. Biological signs of inflammatory disease are frequent. Rheumatoid factor tests are always negative but antinuclear antibodies may be present.

For most diseases, symptoms will vary from person to person. People with the same disease may not have all the symptoms listed. 80%-99% of people have these symptoms

• Antinuclear antibody positivity
• Apraxia
• Arthralgia(Joint pain)
• Dental malocclusion(Bad bite)
• Elevated C-reactive protein level
• Elevated erythrocyte sedimentation rate(High ESR)
• Joint dislocation(Joint dislocations)
• Joint swelling
• Juvenile rheumatoid arthritis
• Limitation of joint mobility(Decreased joint mobility)

Diagnosis

The diagnostic criteria for the disease were established in 2001 at the last international meeting in Edmonton. Rheumatoid factor-negative polyarthritis is defined as the presence of arthritis affecting five or more joints at disease onset. Exclusion criteria are the presence of systemic arthritis or psoriasis in the patient, or a family history of psoriasis in one of the parents or a first-degree relative, HLA B27-positivity in males with onset of arthritis after 6 years of age, and detection of rheumatoid factor IgM in two test samples taken three months apart. Other exclusion criteria include: the presence of ankylosing spondylarthritis, enthesitis and arthritis, sacroiliitis with an inflammatory enteropathy or acute anterior uveitis in the patient, or a family history of one of these conditions in a parent or first-degree relative.

Differential diagnosis

The differential diagnosis should include other forms of polyarthritis (associated with inflammatory or haemato-oncologic diseases).

Treatment

Patients should be managed in a multidisciplinary manner, incorporating expertise in paediatric rheumatology, physiotherapy, psychologyand infantile orthopaedic care.

Treatment is based on the association nonsteroidal anti-inflammatory agents](NSAIDS), disease-modifying antirheumatic drugs (methotrexate as a first-line treatment and tumour necrosis factor (TNF)-alpha as a second-line treatment) and rehabilitation.

Corticotherapy is used at low doses and as rarely as possible. Intraarticular injection of delayed-action corticoids (triamcinolone hexacetonide) may be recommended in case of persistent arthritis.

Prognosis

The prognosis is difficult to determine due to the heterogeneity of the condition but it appears the percentage of remission is low.

NIH genetic and rare disease info

• NIH info on Rheumatoid factor-negative juvenile idiopathic arthritis

Rheumatoid factor-negative juvenile idiopathic arthritis is a rare disease.

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