Adult-onset Still's disease classification: Difference between revisions
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__NOTOC__ | __NOTOC__ | ||
{{Adult-onset Still’s disease}} | {{Adult-onset Still’s disease}} | ||
{{CMG}} | {{CMG}}; {{AE}} {{HK}} | ||
==Overview== | ==Overview== | ||
Adult-onset Stiil's disease (AOSD) may be classified based on the predominant clinical picture with which the patient presents into [[systemic]] sub-type and chronic [[arthritis]] sub-type. The sub-types differ based on the [[cytokine]] profile, clinical course and response to treatment. | |||
==Classification== | ==Classification== | ||
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=== Classification based on clinical presentation === | === Classification based on clinical presentation === | ||
Adult-onset Stiil's disease (AOSD) may be classified based on the predominant clinical picture with which the patient presents. The following are the sub-types:<ref name="pmid15642140">{{cite journal |vauthors=Villanueva J, Lee S, Giannini EH, Graham TB, Passo MH, Filipovich A, Grom AA |title=Natural killer cell dysfunction is a distinguishing feature of systemic onset juvenile rheumatoid arthritis and macrophage activation syndrome |journal=Arthritis Res. Ther. |volume=7 |issue=1 |pages=R30–7 |date=2005 |pmid=15642140 |pmc=1064882 |doi=10.1186/ar1453 |url=}}</ref><ref name="pmid22605480">{{cite journal |vauthors=Lee SJ, Cho YN, Kim TJ, Park SC, Park DJ, Jin HM, Lee SS, Kee SJ, Kim N, Yoo DH, Park YW |title=Natural killer T cell deficiency in active adult-onset Still's Disease: correlation of deficiency of natural killer T cells with dysfunction of natural killer cells |journal=Arthritis Rheum. |volume=64 |issue=9 |pages=2868–77 |date=September 2012 |pmid=22605480 |doi=10.1002/art.34514 |url=}}</ref> | Adult-onset Stiil's disease (AOSD) may be classified based on the predominant clinical picture with which the patient presents. The following are the sub-types:<ref name="pmid15642140">{{cite journal |vauthors=Villanueva J, Lee S, Giannini EH, Graham TB, Passo MH, Filipovich A, Grom AA |title=Natural killer cell dysfunction is a distinguishing feature of systemic onset juvenile rheumatoid arthritis and macrophage activation syndrome |journal=Arthritis Res. Ther. |volume=7 |issue=1 |pages=R30–7 |date=2005 |pmid=15642140 |pmc=1064882 |doi=10.1186/ar1453 |url=}}</ref><ref name="pmid22605480">{{cite journal |vauthors=Lee SJ, Cho YN, Kim TJ, Park SC, Park DJ, Jin HM, Lee SS, Kee SJ, Kim N, Yoo DH, Park YW |title=Natural killer T cell deficiency in active adult-onset Still's Disease: correlation of deficiency of natural killer T cells with dysfunction of natural killer cells |journal=Arthritis Rheum. |volume=64 |issue=9 |pages=2868–77 |date=September 2012 |pmid=22605480 |doi=10.1002/art.34514 |url=}}</ref> | ||
* ASOD with pre-dominant systemic symptoms | * ASOD with pre-dominant [[systemic]] [[symptoms]] | ||
* ASOD with pre-dominant arthritis | * ASOD with pre-dominant [[arthritis]] | ||
{| class="wikitable" | {| class="wikitable" | ||
! rowspan="4" align="center" style="background:#4479BA; color: #FFFFFF;" + |Adult-onset Still's disease sub-types | ! rowspan="4" align="center" style="background:#4479BA; color: #FFFFFF;" + |Adult-onset Still's disease sub-types | ||
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|- | |- | ||
| | | | ||
* Natural killer (NK) cell dysfunction (marked deficiency) | * [[Natural killer cell|Natural killer (NK) cell]] dysfunction (marked deficiency) | ||
* CD8 type (cytotoxic) T cell dysfunction (marked deficiency) | * [[Cytotoxic T cell|CD8 type (cytotoxic) T cell]] dysfunction (marked deficiency) | ||
* Neutrophil, monocyte and macrophage activation | * [[Neutrophil]], [[monocyte]] and [[macrophage]] activation | ||
| | | | ||
* Interleukin 1 beta | * [[Interleukin 1 beta]] | ||
* Interleukin 18 | * [[Interleukin 18]] | ||
* Interferon alpha and beta | * [[Interferon alpha]] and beta | ||
* Interleukin 4 | * [[Interleukin 4]] | ||
| | | | ||
* | * [[Arthralgia]] | ||
* Myalgia | * [[Myalgia]] | ||
* Hepatitis | * [[Hepatitis]] | ||
* Spleenomegaly | * [[Spleenomegaly]] | ||
* Spiking fever (evening spike) | * Spiking [[fever]] (evening spike) | ||
* Orbital inflammation | * [[Orbit (anatomy)|Orbital]] [[inflammation]] | ||
* Cardiac dysfunction | * [[Cardiac]] dysfunction | ||
* Pulmonary dysfucntion | * [[Pulmonary]] dysfucntion | ||
* Weight loss | * [[Weight loss]] | ||
* Sore throat | * [[Sore throat]] | ||
* Lymphadenopathy | * [[Lymphadenopathy]] | ||
| | | | ||
* Thrombocytopenia | * [[Thrombocytopenia]] | ||
* Hyperferritinemia | * Hyperferritinemia | ||
| | | | ||
* Strong response to interleukin-1 inhibitors | * Strong response to [[Interleukin 1|interleukin-1]] inhibitors | ||
* Weak response to interleukin 18 inhibitors and interferon gamma inhibitors | * Weak response to [[interleukin 18]] inhibitors and [[interferon gamma]] inhibitors | ||
| | | | ||
* Upregulation of genes involved in granulocyte formation | * Upregulation of [[genes]] involved in [[granulocyte]] formation | ||
|- | |- | ||
!Arthritis sub-type | !Arthritis sub-type | ||
| | | | ||
* Natural killer (NK) cell dysfunction (deficiency) | * [[Natural killer cells|Natural killer (NK) cell]] dysfunction (deficiency) | ||
* CD8 type (cytotoxic) T cell dysfunction (deficiency) | * [[Cytotoxic T cell|CD8 type (cytotoxic) T cell]] dysfunction (deficiency) | ||
* Neutrophil, monocyte and macrophage activation | * [[Neutrophil]], [[monocyte]] and [[macrophage]] activation | ||
| | | | ||
* Interleukin 17 | * [[Interleukin 17]] | ||
* Interleukin 23 | * [[Interleukin 23]] | ||
* Interleukin 6 | * [[Interleukin 6]] | ||
* Tumor necrosis factor alpha (TNF-alpha) | * [[Tumor necrosis factor alpha|Tumor necrosis factor alpha (TNF-alpha)]] | ||
| | | | ||
* Involvement of wrist, ankle, elbow joints, proximal interphalyngeal joints (PIP) | * Involvement of [[wrist]], [[ankle]], [[Elbow-joint|elbow]] joints, [[Proximal interphalangeal joints|proximal interphalyngeal joints]] (PIP) | ||
* Arthralgia | * [[Arthralgia]] | ||
* Myalgia | * [[Myalgia]] | ||
* Spiking fever | * Spiking [[fever]] | ||
* Salmon-colored maculopapular rash | * Salmon-colored [[maculopapular]] rash | ||
* Less common systemic and organ involvement | * Less common [[systemic]] and organ involvement | ||
| | | | ||
* Thrombocytosis | * [[Thrombocytosis]] | ||
* Arthritis | * [[Arthritis]] | ||
| | | | ||
* Strong response to interleukin 6 inhibitors | * Strong response to [[interleukin 6]] inhibitors | ||
* Good response to TNF-alpha inhibitors | * Good response to [[Tumor necrosis factor-alpha|TNF-alpha]] inhibitors | ||
* Weak reponse to interleukin 17 inhibitors | * Weak reponse to [[interleukin 17]] inhibitors | ||
| | | | ||
|} | |} | ||
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==References== | ==References== | ||
{{reflist|2}} | {{reflist|2}} | ||
[[Category: Rheumatology]] | |||
Latest revision as of 04:50, 23 April 2018
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Adult-onset Still's disease |
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Differentiating Adult-onset Still’s Disease from other Diseases |
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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]
Overview
Adult-onset Stiil's disease (AOSD) may be classified based on the predominant clinical picture with which the patient presents into systemic sub-type and chronic arthritis sub-type. The sub-types differ based on the cytokine profile, clinical course and response to treatment.
Classification
Classification based on clinical presentation
Adult-onset Stiil's disease (AOSD) may be classified based on the predominant clinical picture with which the patient presents. The following are the sub-types:[1][2]
| Adult-onset Still's disease sub-types | Systemic sub-type | Distinguishing features | |||||
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| Pre-dominant cellular mechanism | Cytokine profile | Clinical presentation | Laboratory findings | Response to treatment | Miscellaneous | ||
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| Arthritis sub-type |
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References
- ↑ Villanueva J, Lee S, Giannini EH, Graham TB, Passo MH, Filipovich A, Grom AA (2005). "Natural killer cell dysfunction is a distinguishing feature of systemic onset juvenile rheumatoid arthritis and macrophage activation syndrome". Arthritis Res. Ther. 7 (1): R30–7. doi:10.1186/ar1453. PMC 1064882. PMID 15642140.
- ↑ Lee SJ, Cho YN, Kim TJ, Park SC, Park DJ, Jin HM, Lee SS, Kee SJ, Kim N, Yoo DH, Park YW (September 2012). "Natural killer T cell deficiency in active adult-onset Still's Disease: correlation of deficiency of natural killer T cells with dysfunction of natural killer cells". Arthritis Rheum. 64 (9): 2868–77. doi:10.1002/art.34514. PMID 22605480.