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Still’s disease

Still’s disease is a variant of juvenile idiopathic arthritis that is characterized by seronegative chronic polyarthritis in association with a systemic inflammatory illness. It was initially described in 1897 by George F. Still, a pathologist. The characteristic features of this illness have subsequently been reported in adults, as detailed by Eric Bywaters in 1971.

  • Systemic illness characterized by quotidian fevers, transient rashes, and an inflammatory polyarthritis.

  • No specific test is diagnostic but a ferritin level >1000 ng/mL is common.

  • Macrophage activation syndrome is a severe, life-threatening complication occurring in 5% to 10% of patients.

  • Nonsteroidal antiinflammatory drugs and corticosteroids control 50% of patients with 33% going into remission.

  • Methotrexate and biologics are required for 50% of patients including the 33% with a chronic disease course.

Sign and Symptoms of Adult-Onset Still’s Disease:

MANIFESTATION

FREQUENCY

Arthralgias

98% to 100%

Fever (>39°C)

83% to 100%

Myalgias

84% to 98%

Arthritis

88% to 94%

Sore throat

50% to 92%

Rash

87% to 90%

Weight loss (>10%)

19% to 76%

Lymphadenopathy

48% to 74%

Splenomegaly

45% to 55%

Pleuritis

23% to 53%

Abdominal pain

9% to 48%

Hepatomegaly

29% to 44%

Pericarditis

24% to 37%

Pneumonitis

9% to 31%

Yamaguchi criteria:

Five or more criteria including two or more major criteria yields a 96% sensitivity and 92% specificity to classify a patient as having AOSD.

  • Major criteria
    • Fever >39 °C for >7 days.
    • Arthralgias or arthritis ≥2 weeks.
    • Characteristic rash.
    • Leukocytosis (≥10,000/μL with ≥80% neutrophils).
  • Minor criteria
    • Sore throat.
    • Lymphadenopathy.
    • Hepatomegaly or splenomegaly.Abnormal
    • Negative rheumatoid factor and antinuclear antibody.


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Web:

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MedicineNet

RareDiseases (NORD)

Healthline

WebMD

Internation Still's Disease Information Center