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A chronic inflammatory disease of variable severity that affects the joints and may involve the connective tissues and viscera.

Juvenile Rheumatoid Arthritis (JRA); Still Disease; Still Chauffard Disease. The International League of Associations of Rheumatologists Task Force on the Classification of Childhood Arthritis has proposed that the previously used terminology of juvenile rheumatoid arthritis be discarded and superseded by the umbrella term juvenile idiopathic arthritis (JIA).

Approximately 10-20:100,000 children. A particularly high incidence has been described in Native Americans and in parts of Scandinavia. No sexual predilection exists, however, the oligoarticular and polyarticular forms tend to be more common in girls, whereas the systemic-onset form of the disease affects both genders equally.

A complex genetic inheritance pattern is likely but is yet undefined.

The disease is caused by chronic synovial inflammation of unknown origin characterized by B-lymphocyte infiltration. Synovial proliferation seems to be the result of cytokines released by invading macrophages and T cells. Lymphocytic infiltration has been suggested to induce angiogenesis, thereby maintaining the disease process. Destruction of the joint cartilage and, later, of the bone is caused by the pannus. The etiology of the condition is unclear, but associations with particular HLA haplotypes (e.g., HLA-B27) have been observed.

The previous American College of Rheumatology classification of JRA referred to oligoarticular arthritis, polyarticular arthritis, or systemic illness of at least 6 weeks' duration. In the current classification, JIA is defined as an arthritis occurring before 16 years of age, lasting for at least 6 weeks without an established cause and excludes psoriatic arthropathy and juvenile spondyloarthropathy. The diagnosis is made by exclusion of alternative conditions (e.g., malignancy or infection). The clinical course is variable and may resolve after a single episode, whereas systemic and articular flare-ups occur frequently in others.

The polyarticular form of Chauffard syndrome may occur in children at any age. The oligoarticular and the systemic-onset forms usually occur in early childhood, although later onset (including adolescence) has been described. Skeletal features include generalized osteopenia, joint involvement, either oligoarticular (1-4 joints, also called pauciarticular) or polyarticular (≥5 joints), with cartilage destruction preceding the bony involvement. Effusions in affected joints and radial deviation of the wrist may occur. Joint inflammation may be associated with muscle atrophy. Bony fusion of the cervical spine is common and most frequently occurs at the C2-C3 level secondary to apophyseal ankylosis. Atlantoodontoid instability may occur secondary to pannus formation in the joint, odontoid erosions, and ligamentous laxity. Dental malocclusion and mandibular hypoplasia associated with mandibular retrognathia are common and present in about one fourth of affected children. Limited mouth opening may occur in the absence of symptoms such as temporomandibular joint pain at rest or during function. Stridor may occur secondary to cricoarytenoiditis. Other findings may include uveitis, pericarditis, hepatosplenomegaly, lymphadenopathy, a pink, linear macular rash on trunk and extremities, anemia, and pulmonary interstitial fibrosis. Systemic features such as fever ...

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