Still’s disease

Published on 02/04/2015 by admin

Filed under Internal Medicine

Last modified 22/04/2025

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135 Still’s disease

Instruction

Examine this patient’s joints.

Look at this patient.

Salient features

History

• Maculopapular rash

• History of fever in the initial stages of the disease

• Joint pains, swelling, determine onset and course

• Sore throat.

Examination

• Micrognathia

• Joints usually involved are upper cervical apophyseal joints, carpometacarpal joints and terminal interphalangeal joints (Fig. 135.1)

• Splenomegaly and lymph node enlargement

• Eyes (Fig. 135.2).

image

Fig. 135.1 Hands and wrists in a girl with rheumatoid factor-negative polyarticular juvenile rheumatoid arthritis. There is symmetric involvement of the metacarpophalangeal, proximal interphalangeal and distal interphalangeal joints.

(With permission from Kliegman et al. 2007.)

image

Fig. 135.2 Chronic anterior uveitis in juvenile rheumatoid arthritis, with small irregular pupil, well-developed cataract and early band keratopathy at the medial and lateral margins of the cornea.

(With permission from Kliegman et al. 2007.)

Diagnosis

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

Notes

• The classic triad of Still’s disease is fever, oligoarticular arthritis and an evanescent salmon-coloured rash (which typically appears with the fever spikes), although the rash is persistent in up to one-third of patients.

• A sore throat is characteristic.

• Elevated ferritin levels, leukocyte counts, serum liver enzyme, and ESR are common.

• Still’s disease usually occurs in patients younger than 35 years of age.

Advanced-level questions

What do you know about juvenile chronic arthritis?

The diagnosis is made when a child aged <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 temperature spikes to 39.4°C (103°F) for at least 2 weeks with or without maculopapular 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.

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

What are the complications of juvenile chronic arthritis?

• Pain, lethargy, anorexia and irritability

• Joint contractures

• Anaemia

• Chronic anterior uveitis (iridocyclitis): extensive posterior synechiae result in a small, irregular pupil

• Growth disturbance

• Amyloidosis

• Joint failure.

How would you treat a patient with juvenile chronic arthritis?

• 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, hydrotherapy, splinting

• NSAIDs: naproxen, ibuprofen, piroxicam, indomethacin, diclofenac

• Intra-articular steroids: triamcinolone, methylprednisolone acetate.

What is the risk of treating young children with salicylates?

Reye 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 (Pediatrics 1980;66:859).

What are the poor prognostic factors?

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

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

Which drugs have been used in the treatment of resistant juvenile rheumatoid arthritis?

• Pencillamine

• Hydroxychloroquine

• Methotrexate (long-term therapy should be avoided as it is known to cause hepatic fibrosis)

• Etanercept, adalimumab therapy

• Leflunomide, an oral inhibitor of pyrimidine synthesis.

What do you know about the American College of Rheumatology Pedi Score?

The American College of Rheumatology Pediatric core set of disease-activity measures (ACR Pedi 30) in children with juvenile rheumatoid arthritis consists of:

• a count of swollen joints

• a count of joints with active arthritis

• global assessment of disease activity by the patient or a parent or guardian on a visual-analogue scale ranging from 0 (disease inactive) to 100 (maximal disease activity)

• physician’s global assessment of disease activity

• assessment of physical function by the patient or a parent or guardian (by means of the Childhood Health Assessment Questionnaire Disability Index)

• laboratory evaluation of acute-phase reactants (ESR).

Patients with an ACR Pedi 30 response could also have worsening of 30% or more in no more than 1 of the 6 response variables. The Percent Improvement Index, a continuous variable, is calculated as the mean of the percentage changes from baseline in each core set of disease-activity measures for juvenile rheumatoid arthritis, with negative values indicating improvement and positive values set to 0, indicating no improvement.

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. On a form of chronic joint disease in childhood. Med Chir Trans 1897;80:47). The rash, however, was first described by Eric GL Bywaters.

RDK Reye (1912–1977), an Australian histopathologist.

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