Adult-onset Still's disease classification

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]

ASOD with pre-dominant systemic symptoms
ASOD with pre-dominant arthritis

Systemic sub-type	Distinguishing features
	Pre-dominant cellular mechanism	Cytokine profile	Clinical presentation	Laboratory findings	Response to treatment	Miscellaneous
	Natural killer (NK) cell dysfunction (marked 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	Arthralgia 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

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.

[pmid15642140-1] 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.

[pmid22605480-2] 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.

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