Still's Disease

Examine this patient's joints. Look at this patient.

· Micrognathia.

· Joints usually involved are upper cervical apophyseal joints, carpometacarpal joints and terminal interphalangeal joints.

· Splenomegaly and lymph node enlargement.

This patient has micrognathia, arthropathy of terminal interphalangeal joints and a past history of maculopapular rash (lesion) due to Still's disease (aetiology).

Read BMJ 1995; 310: 728-31.

The diagnosis is made when a child aged less than 16 years has arthritis for at least 6 weeks with no other apparent cause. After 6 months of disease three major patterns are seen:

· Still's disease (10 20% of cases) is defined as arthritis associated with daily tem-perature spikes to 39.4°C (103°F) for at least 2 weeks with or without macule-papular rash.

· Polyarticular juvenile chronic arthritis (15-25%) in which five or more joints are affected. Early fusion of the mandible and cervical spine result in a receding chin and early fusion of the cervical spine.

· Pauciarticular juvenile chronic arthritis (60-75%) which affects four or less joints; iritis is common in girls, whereas sacroiliitis is common in boys.

· Pain, lethargy, anorexia and irritability.

· Joint contractures.

· Anaemia.

· Chronic anterior uveitis.

· Growth disturbance.

· Amyloidosis.

· Joint failure.

· Education about the disease, counselling and social support.

· Physical and occupational therapy: monitoring and recording range of movement of joints, exercises to increase range of movement and muscle bulk, hydro-therapy, splinting.

* NSAIDs: naproxen, ibuprofen, piroxicam, indometacin (indomethacin), diclofenac.

· Intra-articular steroids: triamcinolone, methylprednisolone acetate.

Reye's syndrome has been reported in children treated with aspirin for fever accompanying viral infections such as influenza. Children with juvenile chronic arthritis who are treated continuously for long periods have not been shown to have an increased incidence of this syndrome (Pediatrics1980; 66: 859).

· Chronic and polyarticular arthritis, particularly in patients with a systemic or pauciarticular onset.

· Polyarticular onset and a positive test for immunoglobulin rheumatoid factor.

· Penicillamine.

· Hydroxychloroquine.

· Methotrexate (long-term therapy should be avoided as it is known to cause hepatic fibrosis) (N Engl J Med 1992; 326: 107'7).

Sir George Fredrick Still (1868-1941), a London physician, described 12 children (in 1897) who had a polyarthritis which he stated should be distinguished from rheumatoid arthritis, and a further six children with a disease indistinguishable from adult rheumatoid arthritis. The distinctive findings in the first group included splenomegaly, lymphadenopathy, frequent occurrence of pericarditis and a predilection for cervical spine involvement. He also noted that fever and growth retardation were prominent features (Still GF 1897 On a form of chronic joint disease in childhood.

Med Chir Trans 80: 47). The rash, however, was first described by Eric G.L. Bywaters.

R.D.K. Reye (1912-1977), an Australian histopathologist.