Adult-onset Still's disease

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Adult-onset Still's disease
Classification and external resources
ICD-10 M06.1
ICD-9 714.2
DiseasesDB 34295
MedlinePlus 000450
MeSH D016706

Adult-onset Still's disease is a systemic inflammatory disease. The classic presentation is the triad of persistent high spiking fever, joint pain and a distinctive salmon-colored rash. Serum ferritin, a protein that binds iron, is elevated. There are no characteristic antibodies in the blood. The symptoms are similar to other inflammatory diseases and to autoimmune diseases which do have characteristic antibodies, which must be ruled out with tests for those antibodies. Prognosis is usually favorable but pulmonary, cardiovascular, and kidney manifestations may occasionally cause severe life-threatening complications.[1] It is treated first with steroids such as prednisone. Drugs that block the action of Interleukin-1, particularly IL-1β, are effective in treating it.

Pathophysiology[edit]

The cause of adult-onset Still's disease is not known, but it presumably involves IL-1, since drugs that block the action of IL-1β are effective treatments. Interleukin-18 is expressed at high levels.[1][2]

Signs and symptoms[edit]

The disease typically presents with arthralgia, fever, a 'salmon-pink' rash, and lymphadenopathy. Rheumatoid factor (RF) and anti-nuclear antibody (ANA) are classically negative and serum ferritin is elevated. Patients experiencing a flare-up from Adult-onset Still's disease usually report extreme fatigue, swelling of the lymph glands, and less commonly fluid accumulation in the lungs and heart.

Diagnosis[edit]

The diagnosis is clinical, not based upon serology.[3] 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.[4]

Major criteria Minor criteria
Fever of at least 39C 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

Treatment[edit]

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. Chronic articular disease and polyarticular disease were at higher risk to develop disabling arthritis.[5]

Adult-onset Still's disease is treated with anti-inflammatory drugs. Steroids such as prednisone are used to treat severe symptoms of Still's. Other commonly used medications include hydroxychloroquine, penicillamine, azathioprine, methotrexate, etanercept, anakinra, cyclophosphamide, adalimumab, rituximab, and infliximab.

Newer drugs target interleukin-1 (IL-1), particularly IL-1β. 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. [6] Other anti-IL1β drugs are being developed, including canakinumab and rilonacept.[7]

The condition "juvenile-onset Still's disease" is now usually grouped under juvenile rheumatoid arthritis. However, there is some evidence that the two conditions are closely related.[8]

Epidemiology[edit]

Adult-onset Still's Disease is rare and has been described all over the world. Prevalence is estimated at 1.5 cases per 100,000-1,000,000 population. There is a bimodal age distribution with one peak incidence between ages 15–25 and a second peak between ages of 36–46 years.[9]

History[edit]

Still's disease is named after English physician Sir George Frederic Still (1861–1941).[10][11] It was characterized by EG Bywaters in 1971.[12]

See also[edit]

References[edit]

  1. ^ a b Colafrancesco, Serena; Priori, Roberta; Alessandri, Cristiano; Perricone, Carlo; Pendolino, Monica; Picarelli, Giovanna; Valesini, Guido (2012). "IL-18 Serum Level in Adult Onset Still’s Disease: A Marker of Disease Activity". International Journal of Inflammation 2012: 1–6. doi:10.1155/2012/156890. PMC 3385601. 
  2. ^ Sugiura, T; Kawaguchi, Y; Harigai, M Terajima-Ichida, H; Kitamura, Y; Furuya, T; Ichikawa, N; Kotake, S; Tanaka, M; Hara, M; Kamatani, N (Nov 2002). "Association between adult-onset Still's disease and interleukin-18 gene polymorphisms.". Genes and immunity 3 (7): 394–9. doi:10.1038/sj.gene.6363922. PMID 12424620. 
  3. ^ Efthimiou P, Kontzias A, Ward CM, Ogden NS (June 2007). "Adult-onset Still's disease: can recent advances in our understanding of its pathogenesis lead to targeted therapy?". Nat Clin Pract Rheumatol 3 (6): 328–35. doi:10.1038/ncprheum0510. PMID 17538564. 
  4. ^ Yamaguchi M, Ohta A, Tsunematsu T, Kasukawa R, Mizushima Y, Kashiwagi H, Kashiwazaki S, Tanimoto K, Matsumoto Y, Ota T (1992). "Preliminary criteria for classification of adult Still's disease". J. Rheumatol. 19 (3): 424–30. PMID 1578458. 
  5. ^ Cush, JJ; Medsger TA, Jr; Christy, WC; Herbert, DC; Cooperstein, LA (Feb 1987). "Adult-onset Still's disease. Clinical course and outcome.". Arthritis and rheumatism 30 (2): 186–194. doi:10.1002/art.1780300209. PMID 3827959. 
  6. ^ "Beneficial effect of interleukin 1 inhibition with anakinra in adult-onset Still's disease. An open, randomized, multicenter study.". J. Rheumatol. 39 (10): 2008–11. Oct 2012. doi:10.3899/jrheum.111549. PMID 22859346. 
  7. ^ "Review Article: Anti-Interleukin-1 Agents in Adult Onset Still's Disease". International Journal of Inflammation 2012 (317820). 2012. doi:10.1155/2012/317820. PMID 22611515. 
  8. ^ Luthi F, Zufferey P, Hofer MF, So AK (2002). ""Adolescent-onset Still's disease": characteristics and outcome in comparison with adult-onset Still's disease". Clin. Exp. Rheumatol. 20 (3): 427–30. PMID 12102485. 
  9. ^ Owlia MB, Mehrpoor G. (2009). "Adult - onset Still's disease : A review". Indian J Med Sci 63 (5): 207–21. doi:10.4103/0019-5359.53169. PMID 19584494. 
  10. ^ synd/1773 at Who Named It?
  11. ^ G. F. Still. A special form of joint disease met with in children. Doctoral dissertation, Cambridge, 1896.
  12. ^ Bywaters EG (March 1971). "Still's disease in the adult". Ann. Rheum. Dis. 30 (2): 121–33. doi:10.1136/ard.30.2.121. PMC 1005739. PMID 5315135.