Adult-onset Still's disease

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Adult-onset Still's disease
Sir George Frederic Still

Adult-onset Still's disease (AOSD) is a form of

bumpy rash. The disease is considered a diagnosis of exclusion.[1] Levels of the iron-binding protein ferritin may be extremely elevated with this disorder. AOSD may present in a similar manner to other inflammatory diseases and to autoimmune diseases
, which must be ruled out before making the diagnosis.

Prognosis is usually favorable but manifestations of the disease affecting the lungs, heart, or kidneys may occasionally cause severe life-threatening complications.

interleukin-1, such as anakinra, can be effective treatments when standard steroid treatments are insufficient.[3]

Obvious similarities exist with

juvenile rheumatoid arthritis (also known as "juvenile-onset Still's disease"),[4] and there is some evidence that the two conditions are closely related.[5][6]

Signs and symptoms

The disease typically presents with

serum ferritin is markedly elevated. Patients experiencing a flare-up from adult-onset Still's disease usually report extreme fatigue, swelling of the lymph nodes and, less commonly, fluid accumulation in the lungs and heart. In rare cases, AOSD can cause life-threatening complications, including hemophagocytic lymphohistiocytosis, IVDC, fulminant hepatitis, or disabling conditions such as aseptic meningitis and sensorineural hearing loss.[7][8][9][1]

Pathophysiology

The cause of adult-onset Still's disease is unknown and it is not heritable, but it presumably involves interleukin-1 (IL-1), since medications that block the action of IL-1β are effective treatments.

Interleukin-18 is expressed at high levels.[2][10][11]

Diagnosis

The diagnosis is clinical, not based upon serology.[12] At least seven sets of diagnostic criteria have been devised; however, the Yamaguchi criteria have the highest sensitivity. Diagnosis requires at least five features, with at least two of these being major diagnostic criteria.[13]

Major criteria Minor criteria
Fever of at least 39 °C for at least one week Sore throat
Arthralgias or arthritis for at least two weeks Lymphadenopathy
Nonpruritic salmon-colored rash (usually over trunk or extremities while febrile) Hepatomegaly or splenomegaly
Leukocytosis (10,000/microL or greater), with granulocyte predominance Abnormal liver function tests
Negative tests for antinuclear antibody and rheumatoid factor

Classification

People with AOSD generally experience one of two patterns in the disease:

One set of 21 adult-onset Still's disease patients were divided into four types, according to clinical course patterns. These included monocyclic systemic disease, polycyclic systemic disease, chronic articular monocyclic systemic disease, and chronic articular polycyclic systemic disease. People with chronic articular and polyarticular disease were at higher risk to develop disabling arthritis.[14]

Treatment

Adult-onset Still's disease is treated with anti-inflammatory medications.

Steroids such as prednisone are used to treat severe symptoms of Still's. Other commonly used medications include hydroxychloroquine, penicillamine, azathioprine, methotrexate, etanercept, anakinra, tocilizumab, cyclophosphamide, adalimumab, rituximab, and infliximab.[15]

Newer medications target

interleukin-1 (IL-1), particularly IL-1β.[16] A randomized, multicenter trial reported better outcomes in a group of 12 patients treated with anakinra than in a group of 10 patients taking other disease-modifying antirheumatic drugs.[17] In June 2020 FDA approved Ilaris (canakinumab) for the treatment of AOSD, this is the first FDA approved treatment for AOSD.[18] Canakinumab is another anti-IL1 drug which selectively binds IL-1β and rilonacept which blocks both IL-1A and IL-1β.[19] The monoclonal anti-IL6 antibody tocilizumab is another treatment option as effective as anakinra.[20]

The condition "juvenile-onset Still's disease" is now usually grouped under

juvenile rheumatoid arthritis. However, there are obvious similarities between the two conditions,[4] and there is some evidence that they may be closely related.[5][6]

Epidemiology

Adult-onset Still's disease is rare and has been described all over the world. The number of new cases per year is estimated to be 1.6 per 1,000,000 population.[1] The number of people currently affected is estimated at 1.5 cases per 100,000–1,000,000 population.[citation needed] Onset is most common in two age ranges, between ages 16–25 and between ages of 36–46 years.[21]

History

Still's disease is named after English physician Sir George Frederic Still (1861–1941).[22][23] The adult-onset version was characterized by E. G. Bywaters in 1971.[1]

Research directions

Researchers are investigating whether levels of a protein named calprotectin could be used to improve diagnosis and monitoring.[24]

See also

References

  1. ^
    S2CID 19431424
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  2. ^
    PMID 22762008
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  3. ^
    PMID 24657513
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  4. ^
    PMID 30218025
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  5. ^
    PMID 31769856
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  6. ^
    PMID 31221454
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  7. PMID 31924392
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  8. S2CID 4895740
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  9. PMID 29642928
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  10. S2CID 10549641
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  11. S2CID 44588159
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  12. S2CID 30465113
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  13. PMID 1578458
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  14. PMID 3827959
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  15. PMID 25653531
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  16. PMID 31769856
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  17. S2CID 207614974
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  18. ^ Commissioner, Office of the (2020-06-16). "FDA Approves First Treatment for Adult Onset Still's Disease, a Severe and Rare Disease". FDA. Retrieved 2020-06-21.
  19. PMID 22611515
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  20. PMID 23864171
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  21. PMID 19584494
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  22. Who Named It?
  23. ^ G. F. Still. A special form of joint disease met with in children. Doctoral dissertation, Cambridge, 1896.
  24. PMID 28115781
    .

External links
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