Adult-onset Still's disease classification: Difference between revisions
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* ASOD with pre-dominant arthritis | * ASOD with pre-dominant arthritis | ||
{| class="wikitable" | {| class="wikitable" | ||
! rowspan=" | ! rowspan="4" |Adult-onset Still's disease sub-types | ||
! rowspan="3" |Systemic sub-type | ! rowspan="3" |Systemic sub-type | ||
! colspan=" | ! colspan="6" |Distinguishing features | ||
|- | |- | ||
!Pre-dominant cellular mechanism | !Pre-dominant cellular mechanism | ||
!Cytokine profile | |||
!Clinical presentation | !Clinical presentation | ||
! | !Laboratory findings | ||
!Response to treatment | !Response to treatment | ||
!Miscellaneous | !Miscellaneous | ||
|- | |- | ||
| | | | ||
* Natural killer (NK) cell dysfunction (deficiency) | * Natural killer (NK) cell dysfunction (marked deficiency) | ||
* CD8 type (cytotoxic) T cell dysfunction (deficiency) | * CD8 type (cytotoxic) T cell dysfunction (marked deficiency) | ||
* Neutrophil, monocyte and macrophage activation | |||
| | |||
* Interleukin 1 beta | |||
* Interleukin 18 | |||
* Interferon alpha and beta | |||
* Interleukin 4 | |||
| | | | ||
* Arhtraligia | |||
* Myalgia | |||
* Hepatitis | |||
* Spleenomegaly | |||
* Spiking fever (evening spike) | |||
* Orbital inflammation | |||
* Cardiac dysfunction | |||
* Pulmonary dysfucntion | |||
* Weight loss | |||
* Sore throat | |||
* Lymphadenopathy | |||
| | | | ||
* Thrombocytopenia | |||
* Hyperferritinemia | |||
| | | | ||
* Strong response to 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 (NK) cell dysfunction (deficiency) | |||
* CD8 type (cytotoxic) T cell dysfunction (deficiency) | |||
* Neutrophil, monocyte and macrophage activation | |||
| | | | ||
* Interleukin 17 | |||
* Interleukin 23 | |||
* Interleukin 6 | |||
* Tumor necrosis factor alpha (TNF-alpha) | |||
| | | | ||
* Involvement of wrist, ankle, elbow 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 TNF-alpha inhibitors | |||
* Weak reponse to interleukin 17 inhibitors | |||
| | | | ||
|} | |} | ||
Revision as of 23:55, 7 April 2018
Template:Adult-onset Still's disease
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Overview
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]
- ASOD with pre-dominant systemic symptoms
- ASOD with pre-dominant arthritis
| 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.