Adult-onset Still's disease: Difference between revisions

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'''Adult-onset Still's disease'''
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[[Adult-onset Still's disease|Home]]
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[[Adult-onset Still's disease (patient information)|Patient Information]]
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[[Adult-onset Still's disease overview|Overview]]
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[[Adult-onset Still's disease historical perspective|Historical Perspective]]
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[[Adult-onset Still's disease classification|Classification]]
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[[Adult-onset Still's disease pathophysiology|Pathophysiology]]
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[[Adult-onset Still's disease causes|Causes]]
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[[Adult-onset Still's disease differential diagnosis|Differentiating Xyz from other Diseases]]
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[[Adult-onset Still's disease epidemiology and demographics|Epidemiology and Demographics]]
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[[Adult-onset Still's disease risk factors|Risk Factors]]
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[[Adult-onset Still's disease screening|Screening]]
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[[Adult-onset Still's disease natural history, complications and prognosis|Natural History, Complications and Prognosis]]
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Diagnosis
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[[Adult-onset Still's disease diagnostic study of choice|Diagnostic Study of Choice]]
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[[Adult-onset Still's disease history and symptoms|History and Symptoms]]
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[[Adult-onset Still's disease physical examination|Physical Examination]]
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{{SK}}
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==Overview==
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[[Adult-onset Still's disease laboratory findings|Laboratory Findings]]
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'''Adult-onset Still's disease''' is a rare form of inflammatory [[arthritis]] that was characterized by EG Bywaters in 1971.<ref name="pmid5315135">{{cite journal |author=Bywaters EG |title=Still's disease in the adult |journal=Ann. Rheum. Dis. |volume=30 |issue=2 |pages=121–33 |year=1971 |month=March |pmid=5315135 |pmc=1005739 |doi= 10.1136/ard.30.2.121|url=http://ard.bmj.com/cgi/pmidlookup?view=long&pmid=5315135}}</ref>  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.
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[[Adult-onset Still's disease electrocardiogram|Electrocardiogram]]
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== Historical Perspective ==
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[[Adult-onset Still's disease x ray|X-ray]]
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Still's disease is named after [[United Kingdom|English]] [[physician]] [[Sir George Frederic Still]] (1861–1941).<ref>{{WhoNamedIt|synd|1773}}</ref><ref>G. F. Still. A special form of joint disease met with in children. Doctoral dissertation, Cambridge, 1896.</ref>
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[[Adult-onset Still's disease echocardiography and ultrasound|Echocardiography and Ultrasound]]
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==Classification==
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Adult-onset Still's disease is chronic.
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[[Adult-onset Still's disease CT scan|CT scan]]
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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>
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[[Adult-onset Still's disease MRI|MRI]]
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==Causes==
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[[Adult-onset Still's disease other imaging findings|Other Imaging Findings]]
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== Pathophysiology ==
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[[Adult-onset Still's disease other diagnostic studies|Other Diagnostic Studies]]
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Its pathophysiology is cryptogenic: the underlying cause is unknown.
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Treatment
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==Differentiating Adult-onset Still's Disease from other Diseases==
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[[Adult-onset Still's disease medical therapy|Medical Therapy]]
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== Epidemiology and Demographics ==
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[[Adult-onset Still's disease surgery|Surgery]]
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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.<ref name="Owlia">{{cite journal |author=Owlia MB, Mehrpoor G. |title= Adult - onset Still's disease : A review|journal=Indian J Med Sci |volume=63 | issue = 5 | pages=207–21 |year=2009  |pmid=19584494|doi=10.4103/0019-5359.53169}}</ref>
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[[Adult-onset Still's disease primary prevention|Primary Prevention]]
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===Age===
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[[Adult-onset Still's disease secondary prevention|Secondary Prevention]]
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===Gender===
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[[Adult-onset Still's disease cost-effectiveness of therapy|Cost-Effectiveness of Therapy]]
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===Race===
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[[Adult-onset Still's disease future or investigational therapies|Future or Investigational Therapies]]
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==Risk Factors==
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Case Studies
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==Natural History, Complications and Prognosis==
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[[Adult-onset Still's disease case study one|Case #1]]
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==Diagnosis==
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{{Adult-onset Still's disease}} On the Web
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==Diagnostic Criteria ==
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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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[http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?CMD=search&db=pubmed&term={{urlencode:{{#if:{{{1|}}}|{{{1}}}|{{Adult-onset Still's disease}}}}}} Most recent articles]
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! Major criteria !! Minor criteria
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| Fever of at least 39C for at least one week|| Sore throat
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| Arthralgias or arthritis for at least two weeks|| Lymphadenopathy
[http://scholar.google.com/scholar?ie=UTF-8&oe=utf-8&q={{urlencode:{{#if:{{{1|}}}|{{{1}}}|{{Adult-onset Still's disease}}}}}}&qt_s=Search&sa=N&tab=ps Most cited articles]
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| Nonpruritic salmon colored rash (usually over trunk or extremities while febrile)|| Hepatomegaly or splenomegaly
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| Leukocytosis ( 10,000/microL or greater), with granulocyte predominance|| Abnormal liver function tests
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| || Negative tests for antinuclear antibody and rheumatoid factor
[http://www.ncbi.nlm.nih.gov/sites/entrez?cmd=search&db=pubmed&term={{urlencode:{{#if:{{{1|}}}|{{{1}}}|{{PAGENAME}}}}}}%20AND%20systematic%5Bsb%5D  Review articles]
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[http://www.google.com/search?hl=en&client=firefox-a&rls=org.mozilla:en-US:official&q={{urlencode:{{#if:{{{1|}}}|{{{1}}}|{{PAGENAME}}}}}}+more:health_continuing_education&cx=disease_for_health_professionals&sa=N&oi=coopctx&resnum=0&ct=col3&cd=2 CME Programs]
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===Symptoms===
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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.
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[http://www.google.com/search?q={{urlencode:{{#if:{{{1|}}}|{{{1}}}|{{Adult-onset Still's disease}}}}}}+ppt&ie=utf-8&oe=utf-8&aq=t&rls=org.mozilla:en-US:official&client=firefox-a Powerpoint slides]
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===Physical Examination===
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[http://images.google.com/images?q={{urlencode:{{#if:{{{1|}}}|{{{1}}}|{{Adult-onset Still's disease}}}}}}&ie=UTF-8&oe=utf-8&rls=org.mozilla:en-US:official&client=firefox-a&um=1&sa=N&tab=wi Images]
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===Laboratory Findings===
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American Roentgen Ray Society Images of {{Adult-onset Still's disease}}
:[http://goldminer.arrs.org/search.php?query={{urlencode:{{#if:{{{1|}}}|{{{1}}}|{{Adult-onset Still's disease}}}}}} All Images]
:[http://goldminer.arrs.org/search.php?query={{urlencode:{{#if:{{{1|}}}|{{{1}}}|{{Adult-onset Still's disease}} X-ray}}}} X-rays]
:[http://goldminer.arrs.org/search.php?query={{urlencode:{{#if:{{{1|}}}|{{{1}}}|{{Adult-onset Still's disease}} Ultrasound}}}} Echo & Ultrasound]
:[http://goldminer.arrs.org/search.php?query={{urlencode:{{#if:{{{1|}}}|{{{1}}}|{{Adult-onset Still's disease}} CT}}}} CT Images]
:[http://goldminer.arrs.org/search.php?query={{urlencode:{{#if:{{{1|}}}|{{{1}}}|{{Adult-onset Still's disease}} MRI}}}} MRI]
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===Imaging Findings===
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[http://clinicaltrials.gov/search/open/condition={{urlencode:{{#if:{{{1|}}}|{{{1}}}|{{Adult-onset Still's disease}}}}}} Ongoing Trials  at Clinical Trials.gov]
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===Other Diagnostic Studies===
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[http://www.guideline.gov/search/searchresults.aspx?Type=3&txtSearch={{urlencode:{{#if:{{{1|}}}|{{{1}}}|{{Adult-onset Still's disease}}}}}}&num=20 US National Guidelines Clearinghouse]
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[http://www.nice.org.uk/search/guidancesearchresults.jsp?keywords={{urlencode:{{#if:{{{1|}}}|{{{1}}}|{{Adult-onset Still's disease}}}}}}&searchType=guidance NICE Guidance]
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== Treatment ==
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===Medical Therapy===
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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]].
[http://google2.fda.gov/search?q={{urlencode:{{#if:{{{1|}}}|{{{1}}}|{{Adult-onset Still's disease}}}}}}&x=0&y=0&client=FDA&site=FDA&lr=&proxystylesheet=FDA&output=xml_no_dtd&getfields=* FDA on {{PAGENAME}}]
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===Surgery===
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[http://www.cdc.gov/search.do?queryText={{urlencode:{{#if:{{{1|}}}|{{{1}}}|{{Adult-onset Still's disease}}}}}}&searchButton.x=33&searchButton.y=6&action=search CDC on {{Adult-onset Still's disease}}]
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===Prevention===
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[http://news.google.com/news?q={{urlencode:{{#if:{{{1|}}}|{{{1}}}|{{Adult-onset Still's disease}}}}}}&ie=UTF-8&oe=utf-8&rls=org.mozilla:en-US:official&client=firefox-a&um=1&sa=N&tab=wn {{Adult-onset Still's disease}} in the news]
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==References==
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{{Reflist|2}}
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[http://blogsearch.google.com/blogsearch?q={{urlencode:{{#if:{{{1|}}}|{{{1}}}|{{Adult-onset Still's disease}}}}}}&ie=UTF-8&oe=utf-8&rls=org.mozilla:en-US:official&client=firefox-a&um=1&sa=N&tab=wb Blogs on {{Adult-onset Still's disease}}]
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{{Diseases of the musculoskeletal system and connective tissue}}
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[http://www.google.com/search?hl=en&client=firefox-a&rls=org.mozilla%3Aen-US%3Aofficial&hs=QWo&q={{urlencode:{{#if:{{{1|}}}|{{{1}}}|{{PAGENAME}}}}}}+AND+risk+score+OR+risk+calculator&btnG=Search Risk calculators and risk factors for {{Adult-onset Still's disease}}]
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[[Category:Arthritis]]
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[[Category:Rare diseases]]
[[Category:Disease]]
[[es:Enfermedad de Still]]
[[ko:성인형 스틸병]]
[[it:Malattia di Still dell'adulto]]
[[ja:成人スティル病]]
[[pt:Doença de Still do adulto]]
[[sv:Stills sjukdom]]
{{WH}}
{{WS}}

Revision as of 16:37, 7 April 2018