Adult-Onset Still’s Disease: From Pathophysiology to Targeted Therapies

Table 1

Cytokines involved in the pathogenesis of AOSD and their potential associations with clinical/serological manifestations and disease activity. MAS: Macrophage activation syndrome, CRP: C-Reactive Protein, AOSD: Adult Onset Still’s Disease.

Cytokine	Clinical-serological associations
Tumor necrosis factor-𝛼 (TNF-𝛼)	TNF-𝛼 serum concentration is not associated with disease activity [8, 26]. Levels of TNF receptor (TNF-R) correlate with serum levels of CRP
Interleukin-1𝛽 (IL-1𝛽)	Potential marker for disease activity and useful for monitoring the efficacy of treatment [27, 28]
Soluble interleukin-2 receptor (sIL-2R)	Marker of disease activity [8, 29]
Interleukin-6 (IL-6)	Elevation of CRP, Hyperferritinaemia, Salmon-colored rash
	Hepatosplenomegaly, marker of disease activity [29–31]
Interleukin-8 (IL-8)	Prediction of persistent arthritis but not of disease activity [30]
Interleukin-17 (IL-17)	Elevated in active disease and normalization with the appropriate therapy [20]
Interleukin-18 (IL-18)	Elevation of CRP, hyperferritinaemia, neutrophilia, AOSD-related hepatitis
	Marker of disease severity and response to corticosteroids, MAS [30, 32–36]
Interferon-𝛾 (IFN-𝛾)	Elevated levels in AOSD patients but no correlation with disease activity [26, 29]