Adult-onset Still's disease: Difference between revisions

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__NOTOC__
__NOTOC__
{{Infobox disease
{{Adult-onset Still’s disease}}
| Name          = Adult-onset Still's disease
'''For patient information, click [[Adult-onset Still's Disease (patient information)|here]]'''
| Image          =
| Caption        =
| DiseasesDB    = 34295
| ICD10          = {{ICD10|M|06|1|m|05}}
| ICD9          = {{ICD9|714.2}}
| ICDO          =
| OMIM          =
| MedlinePlus    = 000450
| MeshID        = D016706
}}
{{SI}}
{{CMG}}
==Overview==


'''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.
{{CMG}}; {{AE}} {{HK}}


== Historical Perspective ==
{{SK}} Wissler-Fanconi syndrome, AOSD, Still's disease, adult stills disease


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>


== Epidemiology and Demographics ==
==[[Adult-onset Still's disease overview|Overview]]==


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>
==[[Adult-onset Still's disease historical perspective|Historical Perspective]]==


== Pathophysiology ==
==[[Adult-onset Still's disease classification|Classification]]==


Its pathophysiology is [[cryptogenic]]: the underlying cause is unknown.
==[[Adult-onset Still's disease pathophysiology|Pathophysiology]]==


== Signs and symptoms ==
==[[Adult-onset Still's disease causes|Causes]]==
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.


== Diagnosis ==
==[[Adult-onset Still's disease differential diagnosis|Differentiating Adult-onset Still’s Disease from other Diseases]]==
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>
{| class="wikitable"
|-
! 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 ==
==[[Adult-onset Still's disease epidemiology and demographics|Epidemiology and Demographics]]==
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]].


Adult-onset Still's disease is chronic.
==[[Adult-onset Still's disease risk factors|Risk Factors]]==


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>
==[[Adult-onset Still's disease screening|Screening]]==


==References==
==[[Adult-onset Still's disease natural history, complications and prognosis|Natural History, Complications and Prognosis]]==
{{Reflist|2}}


{{Diseases of the musculoskeletal system and connective tissue}}
==Diagnosis==
[[Adult-onset Still's disease diagnostic study of choice|Diagnostic study of choice]] | [[Adult-onset Still's disease history and symptoms|History and Symptoms]] | [[Adult-onset Still's disease physical examination|Physical Examination]] | [[Adult-onset Still's disease laboratory findings|Laboratory Findings]] | [[Adult-onset Still's disease electrocardiogram|Electrocardiogram]] | [[Adult-onset Still's disease x ray|X-Ray Findings]] | [[Adult-onset Still's disease echocardiography and ultrasound|Echocardiography and Ultrasound]] | [[Adult-onset Still's disease CT scan|CT-Scan Findings]] | [[Adult-onset Still's disease MRI|MRI Findings]] | [[Adult-onset Still's disease other imaging findings|Other Imaging Findings]] | [[Adult-onset Still's disease other diagnostic studies|Other Diagnostic Studies]]


[[Category:Arthritis]]
==Treatment==
[[Category:Rare diseases]]
[[Adult-onset Still's disease medical therapy|Medical Therapy]] | [[Adult-onset Still's disease surgery|Surgery]] | [[Adult-onset Still's disease primary prevention|Primary Prevention]] | [[Adult-onset Still's disease secondary prevention|Secondary Prevention]]
[[Category:Disease]]
 
[[es:Enfermedad de Still]]
==Case Studies==
[[ko:성인형 스틸병]]
[[Adult-onset Still’s Disease case study one|Case #1]]
[[it:Malattia di Still dell'adulto]]
 
[[ja:成人スティル病]]
[[Category:Rheumatology]]
[[pt:Doença de Still do adulto]]
[[Category:autoimmune arthritis]]
[[sv:Stills sjukdom]]
{{WH}}
{{WS}}

Latest revision as of 02:00, 5 December 2019

Adult-onset Still's disease

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Syed Hassan A. Kazmi BSc, MD [2]

Synonyms and keywords: Wissler-Fanconi syndrome, AOSD, Still's disease, adult stills disease


Overview

Historical Perspective

Classification

Pathophysiology

Causes

Differentiating Adult-onset Still’s Disease from other Diseases

Epidemiology and Demographics

Risk Factors

Screening

Natural History, Complications and Prognosis

Diagnosis

Diagnostic study of choice | History and Symptoms | Physical Examination | Laboratory Findings | Electrocardiogram | X-Ray Findings | Echocardiography and Ultrasound | CT-Scan Findings | MRI Findings | Other Imaging Findings | Other Diagnostic Studies

Treatment

Medical Therapy | Surgery | Primary Prevention | Secondary Prevention

Case Studies

Case #1

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