Proceed as follows:

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.

Examine this patient's joints.

Examine the locomotor system in this patient.

· Enlargement of the affected joint (compare with the other side).

· Instability of the joint, in particular hypermobility of the joint.

· May be warm, swollen and tender in the early stages.

· Enlargement and crepitus may be present in the later stages.

· Check sensation in the affected limb.

· Tell the examiner that you would like to investigate as follows:

-Do a thorough neurological examination, looking for loss of proprioception,

and/or pain sensation, and vibratory sensation with a 128 Hz tuning fork.

- Examine the urine for sugar (looking for evidence of diabetes mellitus).

-Ask for lancinating pains, check posterior column signs and look for Argyll

Robertson pupil (tabes dorsalis).

-Check for dissociated sensory loss (syringomyelia).

-Test muscle strength.

This patient has Charcot's joint (lesion) due to diabetes mellitus (aetiology) and has marked deformity of the joint with restricted

movement (functional status).

It is a chronic progressive degenerative arthropathy resulting from a disturbance in the sensory innervation of the affected joint. It is a neuropathic arthropathy which represents a complication of various disorders affecting the nervous system. It results in gross deformity, osteoarthrosis and new bone formation from repeated trauma.

· Diabetic neuropathy - affecting tarsal joints, tarsometatarsal, metatarso-phalangeal joints.

· Tabes dorsalis - affecting knee, hip, ankle, lumbar and lower dorsal vertebrae.

· Syringomyelia - affecting shoulder, elbow, cervical vertebra.

· Myelomeningocele - affecting ankle, tarsus.

· Miscellaneous: hereditary sensory neuropathies, peripheral nerve injury, congenital insensitivity to pain, leprosy.

Jean Martin Charcot (1825-1893) was a French neurologist. He was a Professor of Pathology in 1872 and Professor of Nervous Diseases in 1882. The other conditions that bear his name include the following: Charcot's fever (intermittent fever due to cholangitis), Charcot's triad (intention tremor, nystagmus and scanning speech in multiple sclerosis), Charcot-Leyden crystals (seen in the sputum of asthmatics), Charcot-Marie-Tooth disease (peroneal muscular atrophy), Charcot-Wilbrand syndrome (visual agnosia).