Systemic-onset juvenile idiopathic arthritis: Difference between revisions

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{{Infobox medical condition (new)
 
| name           = Systemic-onset juvenile idiopathic arthritis
{{Infobox medical condition
| synonyms       = '''Systemic juvenile idiopathic arthritis'''
| name                   = Systemic-onset juvenile idiopathic arthritis
| image          =
| synonyms               = Still's disease, systemic juvenile idiopathic arthritis
| caption        =
| field                   = [[Rheumatology]]
| pronounce      =
| symptoms               = [[Fever]], [[rash]], [[arthritis]], [[lymphadenopathy]], [[hepatosplenomegaly]]
| field           = pediatrics/rheumatology
| complications           = [[Macrophage activation syndrome]], [[growth retardation]]
| symptoms       =  
| onset                   = Childhood
| complications   =  
| duration               = Chronic
| onset           =  
| causes                 = Unknown
| duration       =
| risks                   = Genetic predisposition
| types          =  
| diagnosis               = Clinical evaluation, [[blood test]]s
| causes         =  
| differential           = [[Infection]], [[malignancy]], other forms of [[juvenile idiopathic arthritis]]
| risks           =  
| treatment               = [[Nonsteroidal anti-inflammatory drug]]s, [[corticosteroid]]s, [[disease-modifying antirheumatic drug]]s, [[biologic therapy]]
| diagnosis       =  
| medication             = [[Methotrexate]], [[etanercept]], [[anakinra]], [[tocilizumab]]
| differential   =
| prognosis               = Variable; can be severe
| prevention      =  
| frequency               = Rare
| treatment       =  
| medication     =  
| prognosis       =  
| frequency       =
| deaths          =  
}}
}}
 
{{Short description|A subtype of juvenile idiopathic arthritis characterized by systemic symptoms}}
'''Systemic-onset juvenile idiopathic arthritis''' or the juvenile onset form of '''Still's disease'''<ref>{{cite web|title=Still's Disease|url=http://www.medicinenet.com/stills_disease/article.htm|website=MedicineNet|accessdate=8 June 2017|language=en}}</ref>) is a type of [[juvenile idiopathic arthritis]] (JIA) with extra-articular manifestations like fever and rash apart from [[arthritis]]. It was originally called '''systemic-onset juvenile rheumatoid arthritis''' or '''Still's disease'''.
'''Systemic-onset juvenile idiopathic arthritis''' (SoJIA) is a subtype of [[juvenile idiopathic arthritis]] (JIA) that is distinguished by its systemic features, including fever, rash, and inflammation of internal organs, in addition to arthritis. It is a rare and complex condition that primarily affects children.
 
==Clinical Features==
Predominantly extra-articular manifestations like high [[fever]]s, rheumatic rash, [[hepatosplenomegaly|enlargement of the liver and spleen]], [[lymphadenopathy|enlargement of the lymph nodes]], and [[anemia]]. Others manifestations include [[pleuritis|inflammation of the pleura]], [[pericarditis|inflammation of the pericardium]], [[myocarditis|inflammation of the heart's muscular tissue]], and [[peritonitis|inflammation of the peritoneum]] are also seen.{{citation needed|date=April 2014}}
Systemic-onset juvenile idiopathic arthritis is characterized by a combination of systemic and articular symptoms. The hallmark features include:
 
* '''Fever''': Patients typically experience a daily fever that spikes once or twice a day, often reaching 39°C (102°F) or higher.
It is sometimes called "juvenile-onset Still's disease", to distinguish it from [[adult-onset Still's disease]]. However, there is some evidence that the two conditions are closely related.<ref name="pmid12102485">{{cite journal |vauthors=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>
* '''Rash''': A transient, salmon-colored rash may appear, often coinciding with fever spikes. The rash is usually non-itchy and can appear on the trunk and extremities.
 
* '''Arthritis''': Joint inflammation is a key feature, affecting one or more joints. The arthritis can be persistent and may lead to joint damage if not adequately treated.
==Presentation==
* '''Lymphadenopathy''': Swollen lymph nodes are common.
Systemic JIA is characterized by arthritis, [[fever]], which typically is higher than the low-grade fever associated with polyarticular and a salmon pink [[rash]]. It accounts for 10-20% of JIA and affects males and females equally, unlike the other two subtypes of JIA, and affects adolescents. It generally involves both large and small joints. Systemic JIA can be challenging to diagnose because the fever and rash come and go. Fever can occur at the same time every day or twice a day (often in late afternoon or evening) with a spontaneous rapid return to baseline (vs. [[septic arthritis]] of continuous fever). The rash often occurs with fever. It is a discrete, salmon-pink macules of different sizes.  It migrates to different locations on skin, rarely persisting in one location more than one hour. The rash is commonly seen on trunk and proximal extremities or over pressure areas.
* '''Hepatosplenomegaly''': Enlargement of the liver and spleen may occur.
 
* '''Serositis''': Inflammation of serous membranes, such as the pleura or pericardium, can lead to pleuritis or pericarditis.
Arthritis is often absent in the first weeks or even 6–8 months into the illness.
==Pathophysiology==
 
The exact cause of systemic-onset juvenile idiopathic arthritis is unknown, but it is believed to involve a combination of genetic and environmental factors. The condition is thought to be an autoinflammatory disease, characterized by dysregulation of the innate immune system. Key cytokines involved in the inflammatory process include [[interleukin-1]] (IL-1), [[interleukin-6]] (IL-6), and [[interleukin-18]] (IL-18).
Systemic JIA may have [[internal organ]] involvement such as hepatosplenomegaly, lymphadenopathy, [[serositis]], [[hepatitis]], or [[tenosynovitis]].{{citation needed|date=April 2014}}
 
A polymorphism in [[macrophage migration inhibitory factor]] has been associated with this condition.<ref name="pmid12746913">{{cite journal  |vauthors=De Benedetti F, Meazza C, Vivarelli M, etal |title=Functional and prognostic relevance of the -173 polymorphism of the macrophage migration inhibitory factor gene in systemic-onset juvenile idiopathic arthritis |journal=Arthritis Rheum. |volume=48 |issue=5 |pages=1398–407 |date=May 2003 |pmid=12746913 |doi=10.1002/art.10882}}</ref>
==Cause==
The cause is unknown but it's thought to be related to environmental, genetic, and hormonal factors.
 
==Diagnosis==
==Diagnosis==
[[Rheumatoid factor]] and [[Antinuclear antibody|ANA]] tests are generally negative in systemic JIA.
Diagnosis of SoJIA is primarily clinical, based on the characteristic symptoms and exclusion of other conditions. Laboratory tests may show elevated inflammatory markers such as [[C-reactive protein]] (CRP) and [[erythrocyte sedimentation rate]] (ESR). Other tests may include:
Lab findings: [[anemia of chronic disease]], [[neutrophilia]], [[thrombocytosis]], elevated [[Acute-phase protein|acute phase reactants]] ([[Erythrocyte sedimentation rate|ESR]], [[C reactive protein|CRP]], [[ferritin]]).
* '''Complete blood count (CBC)''': May show anemia, leukocytosis, and thrombocytosis.
 
* '''Ferritin levels''': Often elevated in SoJIA.
* '''Antinuclear antibody (ANA) and rheumatoid factor (RF)''': Typically negative in SoJIA.
==Treatment==
==Treatment==
Treatment with either [[glucocorticoids]], [[methotrexate]], [[anakinra]], or [[tocilizumab]] has been examined.<ref name="pmid22290637">{{cite journal |last1=DeWitt |first1=Esi Morgan |last2=Kimura |first2=Yukiko |last3=Beukelman |first3=Timothy |last4=Nigrovic |first4=Peter A. |last5=Onel |first5=Karen |last6=Prahalad |first6=Sampath |last7=Schneider |first7=Rayfel |last8=Stoll |first8=Matthew L. |last9=Angeles-Han |first9=Sheila |last10=Milojevic |first10=Diana |last11=Schikler |first11=Kenneth N. |last12=Vehe |first12=Richard K. |last13=Weiss |first13=Jennifer E. |last14=Weiss |first14=Pamela |last15=Ilowite |first15=Norman T. |last16=Wallace |first16=Carol A. |title=Consensus treatment plans for new-onset systemic juvenile idiopathic arthritis |journal=Arthritis Care & Research |date=1 January 2012 |volume=64 |issue=7 |pages=1001–10 |doi=10.1002/acr.21625 |pmid=22290637 |pmc=3368104}}</ref> [[Anakinra]] has been shown to resolve the clinical features of the disease in 87% of patients.<ref name="Vastert2012">{{cite journal |last1=Vastert |first1=Sebastiaan J |last2=De Jager |first2=Wilco |last3=Noordman |first3=Bo |last4=Prakken |first4=Berent J |last5=Wulffraat |first5=Nico M |title=IL-1 receptor antagonist restores IL-18 NK cell axis in systemic JIA |journal=Journal of Translational Medicine |date=1 January 2012 |volume=10 |issue=Suppl 3 |pages=P45 |doi=10.1186/1479-5876-10-S3-P45 |pmc=3508836}}</ref> It also induces remission in half of corticosteroid-resistant patients.<ref name="Wulffraat2008">{{cite journal |last1=Wulffraat |first1=NM |last2=de Jager |first2=W |last3=Prakken |first3=B |last4=Kuis |first4=W |title=Early effects of Anakinra in corticosteroid naïve SOJIA patients |journal=Pediatric Rheumatology |date=1 January 2008 |volume=6 |issue=Suppl 1 |pages=P29 |doi=10.1186/1546-0096-6-S1-P29|pmc=3334087 }}</ref> The results of another study were similar, with half of the patients responding to treatment with Anakinra.<ref name="pmid18438814">{{cite journal |last1=Gattorno |first1=Marco |last2=Piccini |first2=Alessandra |last3=Lasigliè |first3=Denise |last4=Tassi |first4=Sara |last5=Brisca |first5=Giacomo |last6=Carta |first6=Sonia |last7=Delfino |first7=Laura |last8=Ferlito |first8=Francesca |last9=Pelagatti |first9=Maria Antonietta |last10=Caroli |first10=Francesco |last11=Buoncompagni |first11=Antonella |last12=Viola |first12=Stefania |last13=Loy |first13=Anna |last14=Sironi |first14=Marina |last15=Vecchi |first15=Annunciata |last16=Ravelli |first16=Angelo |last17=Martini |first17=Alberto |last18=Rubartelli |first18=Anna |title=The pattern of response to anti–interleukin-1 treatment distinguishes two subsets of patients with systemic-onset juvenile idiopathic arthritis |journal=Arthritis & Rheumatism |date=1 May 2008 |volume=58 |issue=5 |pages=1505–1515 |doi=10.1002/art.23437 |pmid=18438814}}</ref>  [[Canakinumab]], an antibody to
The management of systemic-onset juvenile idiopathic arthritis involves controlling inflammation and preventing joint damage. Treatment options include:
[[interleukin-1 beta]], is indicated for treatment in patients who respond poorly to other treatments.<ref>http://www.ema.europa.eu/docs/en_GB/document_library/EPAR_-_Product_Information/human/001109/WC500031680.pdf</ref>
* '''Nonsteroidal anti-inflammatory drugs (NSAIDs)''': Used to reduce pain and inflammation.
 
* '''Corticosteroids''': May be used for rapid control of severe systemic symptoms.
* '''Disease-modifying antirheumatic drugs (DMARDs)''': Such as [[methotrexate]], may be used for long-term management.
* '''Biologic agents''': Targeted therapies such as [[IL-1 inhibitors]] (e.g., anakinra) and [[IL-6 inhibitors]] (e.g., tocilizumab) have shown efficacy in treating SoJIA.
==Prognosis==
==Prognosis==
25% of cases progress to severe destructive arthritis.<ref name="pmid16645998">{{cite journal |last=Singh-Grewal |first=D. |author2=Schneider, R. |author3=Bayer, N. |author4=Feldman, B. M. |title=Predictors of disease course and remission in systemic juvenile idiopathic arthritis: Significance of early clinical and laboratory features |journal=Arthritis & Rheumatism |date=1 May 2006 |volume=54 |issue=5 |pages=1595–1601 |doi=10.1002/art.21774 |pmid=16645998}}</ref> In the United States, mortality is estimated at about 4% <ref>{{cite journal |last=Hoffman |first=F. |title=Background Information |journal=Roche Group Media Relations}} http://www.roche.com/med-ra-sjia.pdf.pdf</ref> and in Europe, mortality is estimated at 21.7%.<ref>{{cite journal |last=Davies |first=Rebecca |author2=Southwood, T. |author3=Kearsley-Fleet, L. |author4=Lunt, M. |author5=Hyrich, K. |title=Standardized Mortality Rates are Increased in Patients with Severe Juvenile Idiopathic Arthritis |journal=Oxford Journal of Rheumatology |date=2015 |volume=54 |issue=1 |pages=i153 |url=http://rheumatology.oxfordjournals.org/content/54/suppl_1/i153.2}}</ref>
The prognosis of systemic-onset juvenile idiopathic arthritis varies. Some children experience a monophasic course with complete resolution, while others may have a chronic or polycyclic course with persistent arthritis and systemic symptoms. Early and aggressive treatment can improve outcomes and reduce the risk of complications.
 
==Related Pages==
==History==
* [[Juvenile idiopathic arthritis]]
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> It 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 |date=March 1971 |pmid=5315135 |pmc=1005739 |doi= 10.1136/ard.30.2.121|url=http://ard.bmj.com/cgi/pmidlookup?view=long&pmid=5315135}}</ref><ref name="pmid22573189">{{cite journal|last=Cimaz|first=R|author2=Von, Scheven |author3=Hofer, M |title=Systemic-onset juvenile idiopathic arthritis: the changing life of a rare disease|journal=Swiss Medical Weekly|date=9 May 2012|doi=10.4414/smw.2012.13582|pmid=22573189|volume=142|pages=w13582|url=https://serval.unil.ch/resource/serval:BIB_E8DDE80F9FF8.P001/REF.pdf}}</ref>
* [[Autoinflammatory disease]]
 
* [[Interleukin-1]]
==References==
* [[Interleukin-6]]
{{Reflist}}
[[Category:Juvenile arthritis]]
== External links ==
[[Category:Rheumatology]]
{{Medical resources
|  DiseasesDB    = 12430
|  ICD10          = {{ICD10|M|08|2|m|05}}
|  ICD9          = {{ICD9|714.30}}
|  ICDO          =
|  OMIM          = 604302
|  MedlinePlus    = 
|  eMedicineSubj  = 
|  eMedicineTopic = 
|  MeshID        = 
|  Orphanet      = 85414
}}
[[Category:Arthritis]]
[[Category:Pediatrics]]
[[Category:Pediatrics]]
[[Category:Rheumatology]]
[[Category:Connective tissue diseases]]
[[Category:Inflammatory polyarthropathies]]
[[Category:Idiopathic diseases]]
[[Category:Rare diseases]]
{{dictionary-stub1}}
<gallery>
File:Diseases_of_children_(1916)_(14579765477).jpg
</gallery>

Latest revision as of 02:12, 4 April 2025


Systemic-onset juvenile idiopathic arthritis
Synonyms Still's disease, systemic juvenile idiopathic arthritis
Pronounce N/A
Specialty N/A
Symptoms Fever, rash, arthritis, lymphadenopathy, hepatosplenomegaly
Complications Macrophage activation syndrome, growth retardation
Onset Childhood
Duration Chronic
Types N/A
Causes Unknown
Risks Genetic predisposition
Diagnosis Clinical evaluation, blood tests
Differential diagnosis Infection, malignancy, other forms of juvenile idiopathic arthritis
Prevention N/A
Treatment Nonsteroidal anti-inflammatory drugs, corticosteroids, disease-modifying antirheumatic drugs, biologic therapy
Medication Methotrexate, etanercept, anakinra, tocilizumab
Prognosis Variable; can be severe
Frequency Rare
Deaths N/A


A subtype of juvenile idiopathic arthritis characterized by systemic symptoms


Systemic-onset juvenile idiopathic arthritis (SoJIA) is a subtype of juvenile idiopathic arthritis (JIA) that is distinguished by its systemic features, including fever, rash, and inflammation of internal organs, in addition to arthritis. It is a rare and complex condition that primarily affects children.

Clinical Features[edit]

Systemic-onset juvenile idiopathic arthritis is characterized by a combination of systemic and articular symptoms. The hallmark features include:

  • Fever: Patients typically experience a daily fever that spikes once or twice a day, often reaching 39¬∞C (102¬∞F) or higher.
  • Rash: A transient, salmon-colored rash may appear, often coinciding with fever spikes. The rash is usually non-itchy and can appear on the trunk and extremities.
  • Arthritis: Joint inflammation is a key feature, affecting one or more joints. The arthritis can be persistent and may lead to joint damage if not adequately treated.
  • Lymphadenopathy: Swollen lymph nodes are common.
  • Hepatosplenomegaly: Enlargement of the liver and spleen may occur.
  • Serositis: Inflammation of serous membranes, such as the pleura or pericardium, can lead to pleuritis or pericarditis.

Pathophysiology[edit]

The exact cause of systemic-onset juvenile idiopathic arthritis is unknown, but it is believed to involve a combination of genetic and environmental factors. The condition is thought to be an autoinflammatory disease, characterized by dysregulation of the innate immune system. Key cytokines involved in the inflammatory process include interleukin-1 (IL-1), interleukin-6 (IL-6), and interleukin-18 (IL-18).

Diagnosis[edit]

Diagnosis of SoJIA is primarily clinical, based on the characteristic symptoms and exclusion of other conditions. Laboratory tests may show elevated inflammatory markers such as C-reactive protein (CRP) and erythrocyte sedimentation rate (ESR). Other tests may include:

  • Complete blood count (CBC): May show anemia, leukocytosis, and thrombocytosis.
  • Ferritin levels: Often elevated in SoJIA.
  • Antinuclear antibody (ANA) and rheumatoid factor (RF): Typically negative in SoJIA.

Treatment[edit]

The management of systemic-onset juvenile idiopathic arthritis involves controlling inflammation and preventing joint damage. Treatment options include:

  • Nonsteroidal anti-inflammatory drugs (NSAIDs): Used to reduce pain and inflammation.
  • Corticosteroids: May be used for rapid control of severe systemic symptoms.
  • Disease-modifying antirheumatic drugs (DMARDs): Such as methotrexate, may be used for long-term management.
  • Biologic agents: Targeted therapies such as IL-1 inhibitors (e.g., anakinra) and IL-6 inhibitors (e.g., tocilizumab) have shown efficacy in treating SoJIA.

Prognosis[edit]

The prognosis of systemic-onset juvenile idiopathic arthritis varies. Some children experience a monophasic course with complete resolution, while others may have a chronic or polycyclic course with persistent arthritis and systemic symptoms. Early and aggressive treatment can improve outcomes and reduce the risk of complications.

Related Pages[edit]

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