Demographic, clinical, and laboratory characteristics of the patients enrolled in the study
| . | Inactive sJIA (N = 17) . | Active sJIA (N = 27) . | MAS (N = 14) . | iaHLH (N = 7*) . | Other sHLH (N = 9†) . |
|---|---|---|---|---|---|
| Sex, female‡ | 7 (41.2) | 12 (44.4) | 8 (57.1) | 3 (42.9) | 2 (22.2) |
| Age at sampling (y)§ | 8.6 (6.8-13.7) | 9.8 (4.5-12.9) | 11.6 (5.8-15.1) | 2.8 (1.3-5.2) | 7.0 (1.8-9.8) |
| Days from onset of symptoms§ | — | 17.0 (9.5-22.0) | 18.5 (12.0-26.0) | 18.0 (15.0-41.0) | 34.0 (16.0-191.0) |
| Clinical features at sampling | |||||
| Fever‡ | 0 (0.0) | 25 (92.6) | 14 (100.0) | 6 (85.7) | 8 (88.9) |
| Splenomegaly‡ | 0 (0.0) | 6 (22.2) | 8 (57.1) | 5 (71.4) | 6 (66.7) |
| Laboratory features at sampling | |||||
| Ferritin (ng/mL)§ | 32 (22-58) | 738 (326-4 289) | 7 979 (3 298-24 741) | 8 211 (3 267-38 346) | 9 989 (3 597-16 462) |
| CRP (mg/dL)§ | 0.07 (0.03-0.19) | 8.99 (4.66-17.05) | 4.30 (2.32-8.79) | 1.54 (0.92-2.60) | 1.61 (1.15-16.39) |
| WBC (×103/μL)§ | 5.8 (5.2-7.6) | 14.9 (8.2-18.6) | 5.3 (4.2-9.3) | 4.9 (4.0-7.4) | 2.9 (1.1-6.8) |
| Neutrophils (×103/μL)§ | 2.8 (2.3-3.2) | 11.9 (5.2-16.8) | 5.5 (3.0-7.2) | 0.9 (0.6-2.6) | 1.5 (0.8-2.3) |
| Hb (g/dL)§ | 13.2 (11.8-13.7) | 11.0 (10.2-11.8) | 11.0 (10.1-12.7) | 8.7 (8.3-10.0) | 8.8 (8.0-10.1) |
| PLTs (×103/μL)§ | 260 (248-301) | 405 (273-526) | 184 (103-225) | 99 (75-134) | 84 (39-135) |
| AST (U/L)§ | 23 (21-31) | 36 (25-44) | 87 (68-175) | 295 (174-337) | 98 (76-139) |
| LDH (U/L)§ | 323 (218-472) | 506 (377-673) | 1 066 (667-1 506) | 2 840 (2 144-3 973) | 1 389 (913-2 318) |
| Triglycerides (mg/dL)§ | 65 (47-98) | 104 (71-128) | 196 (178-268) | 296 (184-353) | 444 (334-495) |
| Fibrinogen (mg/dL)§ | 259 (237-348) | 635 (509-697) | 344 (311-385) | 143 (123-195) | 308 (123-350) |
| MAS/HLH biomarkers at sampling | |||||
| CXCL9 (pg/mL)§,‖ | 300 (300-582) | 300 (300-300) | 4 753 (2 763-9 750) | 17 133 (5 163-28 023) | 3 089 (2 753-9 103) |
| IL-18 (pg/mL)§,¶ | 11 971 (322-45 750) | 17 505 (4 208-32 279) | 139 121 (46 814-292 061) | 16 941 (286-100 678) | 11 813 (5 593-37 220) |
| Any treatment at sampling‡ | 12 (70.6) | 10 (37.0) | 7 (50.0) | 1 (14.3) | 9 (100.0) |
| Glucocorticoids < 14 d‡ | 0 (0.0) | 5 (18.5) | 6 (42.8) | 1 (0.14) | 3 (33.3) |
| Glucocorticoids ≥ 14 d‡ | 3 (17.6) | 3 (11.1) | 0 (0.0) | 0 (0.0) | 6 (66.7) |
| Anakinra‡ | 11 (64.7) | 1 (3.7) | 2 (14.3) | 0 (0.0) | 3 (33.3) |
| Other treatment‡,# | 1 (5.9) | 3 (11.1) | 1 (7.1) | 1 (14.2) | 4 (44.4) |
| . | Inactive sJIA (N = 17) . | Active sJIA (N = 27) . | MAS (N = 14) . | iaHLH (N = 7*) . | Other sHLH (N = 9†) . |
|---|---|---|---|---|---|
| Sex, female‡ | 7 (41.2) | 12 (44.4) | 8 (57.1) | 3 (42.9) | 2 (22.2) |
| Age at sampling (y)§ | 8.6 (6.8-13.7) | 9.8 (4.5-12.9) | 11.6 (5.8-15.1) | 2.8 (1.3-5.2) | 7.0 (1.8-9.8) |
| Days from onset of symptoms§ | — | 17.0 (9.5-22.0) | 18.5 (12.0-26.0) | 18.0 (15.0-41.0) | 34.0 (16.0-191.0) |
| Clinical features at sampling | |||||
| Fever‡ | 0 (0.0) | 25 (92.6) | 14 (100.0) | 6 (85.7) | 8 (88.9) |
| Splenomegaly‡ | 0 (0.0) | 6 (22.2) | 8 (57.1) | 5 (71.4) | 6 (66.7) |
| Laboratory features at sampling | |||||
| Ferritin (ng/mL)§ | 32 (22-58) | 738 (326-4 289) | 7 979 (3 298-24 741) | 8 211 (3 267-38 346) | 9 989 (3 597-16 462) |
| CRP (mg/dL)§ | 0.07 (0.03-0.19) | 8.99 (4.66-17.05) | 4.30 (2.32-8.79) | 1.54 (0.92-2.60) | 1.61 (1.15-16.39) |
| WBC (×103/μL)§ | 5.8 (5.2-7.6) | 14.9 (8.2-18.6) | 5.3 (4.2-9.3) | 4.9 (4.0-7.4) | 2.9 (1.1-6.8) |
| Neutrophils (×103/μL)§ | 2.8 (2.3-3.2) | 11.9 (5.2-16.8) | 5.5 (3.0-7.2) | 0.9 (0.6-2.6) | 1.5 (0.8-2.3) |
| Hb (g/dL)§ | 13.2 (11.8-13.7) | 11.0 (10.2-11.8) | 11.0 (10.1-12.7) | 8.7 (8.3-10.0) | 8.8 (8.0-10.1) |
| PLTs (×103/μL)§ | 260 (248-301) | 405 (273-526) | 184 (103-225) | 99 (75-134) | 84 (39-135) |
| AST (U/L)§ | 23 (21-31) | 36 (25-44) | 87 (68-175) | 295 (174-337) | 98 (76-139) |
| LDH (U/L)§ | 323 (218-472) | 506 (377-673) | 1 066 (667-1 506) | 2 840 (2 144-3 973) | 1 389 (913-2 318) |
| Triglycerides (mg/dL)§ | 65 (47-98) | 104 (71-128) | 196 (178-268) | 296 (184-353) | 444 (334-495) |
| Fibrinogen (mg/dL)§ | 259 (237-348) | 635 (509-697) | 344 (311-385) | 143 (123-195) | 308 (123-350) |
| MAS/HLH biomarkers at sampling | |||||
| CXCL9 (pg/mL)§,‖ | 300 (300-582) | 300 (300-300) | 4 753 (2 763-9 750) | 17 133 (5 163-28 023) | 3 089 (2 753-9 103) |
| IL-18 (pg/mL)§,¶ | 11 971 (322-45 750) | 17 505 (4 208-32 279) | 139 121 (46 814-292 061) | 16 941 (286-100 678) | 11 813 (5 593-37 220) |
| Any treatment at sampling‡ | 12 (70.6) | 10 (37.0) | 7 (50.0) | 1 (14.3) | 9 (100.0) |
| Glucocorticoids < 14 d‡ | 0 (0.0) | 5 (18.5) | 6 (42.8) | 1 (0.14) | 3 (33.3) |
| Glucocorticoids ≥ 14 d‡ | 3 (17.6) | 3 (11.1) | 0 (0.0) | 0 (0.0) | 6 (66.7) |
| Anakinra‡ | 11 (64.7) | 1 (3.7) | 2 (14.3) | 0 (0.0) | 3 (33.3) |
| Other treatment‡,# | 1 (5.9) | 3 (11.1) | 1 (7.1) | 1 (14.2) | 4 (44.4) |
CRP, C-reactive protein; Hb, hemoglobin; PLTs, platelets; WBC, white blood cells.
Three EBV, 2 leishmania, 1 CMV, and 1 bocavirus.
Two p.R186C CDC42 mutated patients, 1 NLRC4-mutated patient, 2 unknown immune-deficiencies, 2 tumors, 1 Kikuchi Fujimoto disease, and 1 Rasmussen syndrome.
Number (percentage).
Median (first to third quartile).
Median (first to third quartile) in healthy controls in our laboratory, 612 (430-802) pg/mL.
Median (first to third quartile) in healthy controls in our laboratory, 265 (221-305) pg/mL.
Cyclosporine-A (9), tocilizumab (1), canakinumab (1), and emapalumab (1).