Adult-onset Still's disease classification: Difference between revisions
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{{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== | ||
=== 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> | |||
* ASOD with pre-dominant [[systemic]] [[symptoms]] | |||
* ASOD with pre-dominant [[arthritis]] | |||
{| class="wikitable" | |||
! rowspan="4" align="center" style="background:#4479BA; color: #FFFFFF;" + |Adult-onset Still's disease sub-types | |||
! rowspan="3" |Systemic sub-type | |||
! colspan="6" align="center" style="background:#4479BA; color: #FFFFFF;" + |Distinguishing features | |||
|- | |||
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Pre-dominant cellular mechanism | |||
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Cytokine profile | |||
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Clinical presentation | |||
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Laboratory findings | |||
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Response to treatment | |||
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Miscellaneous | |||
|- | |||
| | |||
* [[Natural killer cell|Natural killer (NK) cell]] dysfunction (marked deficiency) | |||
* [[Cytotoxic T cell|CD8 type (cytotoxic) T cell]] dysfunction (marked deficiency) | |||
* [[Neutrophil]], [[monocyte]] and [[macrophage]] activation | |||
| | |||
* [[Interleukin 1 beta]] | |||
* [[Interleukin 18]] | |||
* [[Interferon alpha]] and beta | |||
* [[Interleukin 4]] | |||
| | |||
* [[Arthralgia]] | |||
* [[Myalgia]] | |||
* [[Hepatitis]] | |||
* [[Spleenomegaly]] | |||
* Spiking [[fever]] (evening spike) | |||
* [[Orbit (anatomy)|Orbital]] [[inflammation]] | |||
* [[Cardiac]] dysfunction | |||
* [[Pulmonary]] dysfucntion | |||
* [[Weight loss]] | |||
* [[Sore throat]] | |||
* [[Lymphadenopathy]] | |||
| | |||
* [[Thrombocytopenia]] | |||
* Hyperferritinemia | |||
| | |||
* Strong response to [[Interleukin 1|interleukin-1]] inhibitors | |||
* Weak response to [[interleukin 18]] inhibitors and [[interferon gamma]] inhibitors | |||
| | |||
* Upregulation of [[genes]] involved in [[granulocyte]] formation | |||
|- | |||
!Arthritis sub-type | |||
| | |||
* [[Natural killer cells|Natural killer (NK) cell]] dysfunction (deficiency) | |||
* [[Cytotoxic T cell|CD8 type (cytotoxic) T cell]] dysfunction (deficiency) | |||
* [[Neutrophil]], [[monocyte]] and [[macrophage]] activation | |||
| | |||
* [[Interleukin 17]] | |||
* [[Interleukin 23]] | |||
* [[Interleukin 6]] | |||
* [[Tumor necrosis factor alpha|Tumor necrosis factor alpha (TNF-alpha)]] | |||
| | |||
* Involvement of [[wrist]], [[ankle]], [[Elbow-joint|elbow]] joints, [[Proximal interphalangeal joints|proximal interphalyngeal joints]] (PIP) | |||
* [[Arthralgia]] | |||
* [[Myalgia]] | |||
* Spiking [[fever]] | |||
* Salmon-colored [[maculopapular]] rash | |||
* Less common [[systemic]] and organ involvement | |||
| | |||
* [[Thrombocytosis]] | |||
* [[Arthritis]] | |||
| | |||
* Strong response to [[interleukin 6]] inhibitors | |||
* Good response to [[Tumor necrosis factor-alpha|TNF-alpha]] inhibitors | |||
* Weak reponse to [[interleukin 17]] inhibitors | |||
| | |||
|} | |||
==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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Diagnosis |
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Treatment |
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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.