The role of S100 proteins in the pathogenesis and monitoring of autoinflammatory diseases

Molecular and Cellular Pediatrics

Table 2 Serum concentration of phagocyte-specific S100 proteins in systemic inflammatory diseases (adapted and updated from [15])

	S100A8/A9 levels (ng/ml)	N	S100A12 levels (ng/ml)	N
Healthy controls	340 ± 70	50	50 ± 10	45
			50 (5)**	74
Monogenic autoinflammatory diseases
FMF	110,000 ± 82,000	20	6720 ± 4960	17
FMF	110,000 ± 82,000	20	33,500 (22,200)**	7
PAPA	116,000 ± 74,000	11	–
PAMI	2,045,000 ± 1,300,000	13	–
NOMID	2830 ± 580	18	720 ± 450	18
MWS	4390 (2535)*	12	150 ± 60	17
FCAS	3600 (4610)*	5	–	–
Polygenic autoinflammatory diseases
Systemic-onset JIA	14,920 ± 4030	60	7190 ± 2690	60
Systemic-onset JIA	24,750 ± 11,410	20	3700 (1080)**	33
Polyarticular JIA	2380 ± 530	89	395 (45)**	89
PFAPA	3846 ± 1197	15	685 ± 210	15
Vasculitis
Kawasaki disease	3630 ± 480	21	398 (294)*	67
Henoch-Schoenlein nephritis	881 ± (670)*	30	–	–
Infections
Severe febrile infections	3720 ± 870	66	470 ± 160	83

All other data are mean ± 95% confidence interval
Italics indicate the diseases with the significantly highest S100 protein serum levels
FCAS familial cold autoinflammatory syndrome, FMF familial Mediterranean fever, JIA juvenile idiopathic arthritis, MWS Muckle-Wells syndrome, N number of patients, NOMID Neonatal Onset Multisystem Inflammatory Disorder, PAMI PSTPIP1-associated myeloid-related proteinemia inflammatory, PAPA pyogenic sterile arthritis, pyoderma gangrenosum, and acne syndrome, PFAPA periodic fever, aphthous stomatitis, pharyngitis, adenitis syndrome
*Mean (standard deviation)
**Mean (standard error of the mean)