Adult-onset Still's disease: Difference between revisions
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==Classification== | ==Classification== | ||
Adult-onset Still's disease is chronic. | |||
The condition "juvenile-onset Still disease" is now usually grouped under [[juvenile rheumatoid arthritis]]. However, there is some evidence that the two conditions are closely related.<ref name="pmid12102485">{{cite journal |author=Luthi F, Zufferey P, Hofer MF, So AK |title="Adolescent-onset Still's disease": characteristics and outcome in comparison with adult-onset Still's disease |journal=Clin. Exp. Rheumatol. |volume=20 |issue=3 |pages=427–30 |year=2002 |pmid=12102485 |doi= |url=}}</ref> | |||
==Causes== | ==Causes== | ||
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==Natural History, Complications and Prognosis== | ==Natural History, Complications and Prognosis== | ||
==Diagnosis== | |||
== | ===Diagnostic Criteria == | ||
The diagnosis is clinical, not based upon [[serology]].<ref name="pmid17538564">{{cite journal |author=Efthimiou P, Kontzias A, Ward CM, Ogden NS |title=Adult-onset Still's disease: can recent advances in our understanding of its pathogenesis lead to targeted therapy? |journal=Nat Clin Pract Rheumatol |volume=3 |issue=6 |pages=328–35 |year=2007 |month=June |pmid=17538564 |doi=10.1038/ncprheum0510}}</ref> 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.<ref name="Yamaguchi">{{cite journal |author=Yamaguchi M, Ohta A, Tsunematsu T, Kasukawa R, Mizushima Y, Kashiwagi H, Kashiwazaki S, Tanimoto K, Matsumoto Y, Ota T |title=Preliminary criteria for classification of adult Still's disease |journal=J Rheumatol |volume=19 |issue=3 |pages=424–30 |year=1992 |pmid=1578458 }}</ref> | The diagnosis is clinical, not based upon [[serology]].<ref name="pmid17538564">{{cite journal |author=Efthimiou P, Kontzias A, Ward CM, Ogden NS |title=Adult-onset Still's disease: can recent advances in our understanding of its pathogenesis lead to targeted therapy? |journal=Nat Clin Pract Rheumatol |volume=3 |issue=6 |pages=328–35 |year=2007 |month=June |pmid=17538564 |doi=10.1038/ncprheum0510}}</ref> 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.<ref name="Yamaguchi">{{cite journal |author=Yamaguchi M, Ohta A, Tsunematsu T, Kasukawa R, Mizushima Y, Kashiwagi H, Kashiwazaki S, Tanimoto K, Matsumoto Y, Ota T |title=Preliminary criteria for classification of adult Still's disease |journal=J Rheumatol |volume=19 |issue=3 |pages=424–30 |year=1992 |pmid=1578458 }}</ref> | ||
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| || Negative tests for antinuclear antibody and rheumatoid factor | | || Negative tests for antinuclear antibody and rheumatoid factor | ||
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===Symptoms=== | |||
The disease typically presents with [[arthralgia]], [[fever]], elevated [[serum ferritin]], a 'salmon-pink' rash, [[pyrexia]] and [[lymphadenopathy]]. Rheumatoid factor (RF) and anti-nuclear antibody (ANA) are classically negative. 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. | |||
===Physical Examination=== | |||
===Laboratory Findings=== | |||
===Imaging Findings=== | |||
===Other Diagnostic Studies=== | |||
== Treatment == | == Treatment == | ||
===Medical Therapy=== | |||
Treatment for adult-onset Still's disease is with anti-inflammatory drugs. [[Steroids]] such as [[prednisone]] are used to treat severe symptoms of Still's. Other medications include [[hydroxychloroquine]], [[penicillamine]], [[azathioprine]], [[methotrexate]], [[etanercept]], [[anakinra]], [[cyclophosphamide]], [[adalimumab]], [[rituximab]], and [[infliximab]]. | Treatment for adult-onset Still's disease is with anti-inflammatory drugs. [[Steroids]] such as [[prednisone]] are used to treat severe symptoms of Still's. Other medications include [[hydroxychloroquine]], [[penicillamine]], [[azathioprine]], [[methotrexate]], [[etanercept]], [[anakinra]], [[cyclophosphamide]], [[adalimumab]], [[rituximab]], and [[infliximab]]. | ||
===Surgery=== | |||
===Prevention=== | |||
==References== | ==References== | ||
Revision as of 14:01, 7 June 2016
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Synonyms and keywords:
Overview
Adult-onset Still's disease is a rare form of inflammatory arthritis that was characterized by EG Bywaters in 1971.[1] The underlying cause is unknown. It usually presents with high spiking fevers, joint and muscle pains, a salmon colored rash and other symptoms of systemic inflammation.
Historical Perspective
Still's disease is named after English physician Sir George Frederic Still (1861–1941).[2][3]
Classification
Adult-onset Still's disease is chronic.
The condition "juvenile-onset Still disease" is now usually grouped under juvenile rheumatoid arthritis. However, there is some evidence that the two conditions are closely related.[4]
Causes
Pathophysiology
Its pathophysiology is cryptogenic: the underlying cause is unknown.
Differentiating Adult-onset Still's Disease from other Diseases
Epidemiology and Demographics
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.[5]
Age
Gender
Race
Risk Factors
Natural History, Complications and Prognosis
Diagnosis
=Diagnostic Criteria
The diagnosis is clinical, not based upon serology.[6] 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.[7]
| 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 |
Symptoms
The disease typically presents with arthralgia, fever, elevated serum ferritin, a 'salmon-pink' rash, pyrexia and lymphadenopathy. Rheumatoid factor (RF) and anti-nuclear antibody (ANA) are classically negative. 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.
Physical Examination
Laboratory Findings
Imaging Findings
Other Diagnostic Studies
Treatment
Medical Therapy
Treatment for adult-onset Still's disease is with anti-inflammatory drugs. Steroids such as prednisone are used to treat severe symptoms of Still's. Other medications include hydroxychloroquine, penicillamine, azathioprine, methotrexate, etanercept, anakinra, cyclophosphamide, adalimumab, rituximab, and infliximab.
Surgery
Prevention
References
- ↑ Bywaters EG (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. Unknown parameter
|month=ignored (help) - ↑ Template:WhoNamedIt
- ↑ G. F. Still. A special form of joint disease met with in children. Doctoral dissertation, Cambridge, 1896.
- ↑ 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.
- ↑ 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.
- ↑ Efthimiou P, Kontzias A, Ward CM, Ogden NS (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. Unknown parameter
|month=ignored (help) - ↑ 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.
Template:Diseases of the musculoskeletal system and connective tissue ko:성인형 스틸병 it:Malattia di Still dell'adulto sv:Stills sjukdom Template:WH Template:WS