Table 2. Baseline characteristics of 20 patients with MAS.
| Total (n = 20) | SLE (n = 14) | AOSD (n = 6) | |
|---|---|---|---|
| Sex, female, n (%) | 18 (90) | 13 (92.9) | 5 (83.3) |
| Age, years, mean ± SD | 35.6 ± 18.3 | 37.4±16.2 | 39.7±18.8 |
| Disease duration to MAS occurrence¶, months, mean ± SD | 31.3±56.4 | 44.6±63.4 | 0.2±0.4 |
| Clinical features | |||
| Hepatomegaly, n (%) | 7 (35) | 3 (21.4) | 4 (66.7) |
| Lymphadenopathy, n (%) | 14 (70) | 10 (71.4) | 4 (66.7) |
| Pneumonitis, n (%) | 6 (30) | 5 (35.7) | 1 (16.7) |
| Renal impairment¶¶, n (%) | 6 (30) | 5 (35.7) | 1 (16.7) |
| Neuropsychiatric manifestations, n (%) | 6 (30) | 5 (35.7) | 1 (16.7) |
| Lab findings | |||
| ANC, /uL, median (IQR) | 2890 (875, 8285) | 2544.5 (590.0, 5512.5) | 5420.0 (1605.0, 11877.5) |
| Hb, g/dL, median (IQR) | 8.5 (7.2, 9.0) | 8.6 (7.5, 9.1) | 7.6 (6.5, 9.3) |
| PLT, x 109/L, mean ± SD | 101.3±95.1 | 66.6±30.6 | 182.2±144.0 |
| AST, IU/L, median (IQR) | 137.5 (75.8, 293.0) | 155.0 (91.5, 564.8) | 506.0 (72.8, 919.0) |
| LDH, IU/L, median (IQR) | 899.5 (381.5, 1556.8) | 648.5 (358.0, 1729.8) | 1221.0 (743.5, 3381.3) |
| TB, mg/dL, median (IQR) | 0.8 (0.5, 2.8) | 0.5 (0.3, 3.4) | 1.0 (0.8, 2.7) |
| Albumin, g/dL, mean ± SD | 2.4±0.4 | 2.3±0.4 | 2.5±0.4 |
| eGFR$, mL/min/1.73m2, mean ± SD | 101.8±40.8 | 96.4±42.4 | 114.5±37.6 |
| ESR, mm/hr, median (IQR) | 26.5(17.0, 53.0) | 30.0 (17.8, 59.5) | 21.0 (14.3, 39.0) |
| CRP¶, mg/dL, median (IQR) | 2.2 (0.6, 6.3) | 1.4 (0.3, 3.4) | 6.0 (2.3, 6.6) |
| Ferritin, ng/mL, median (IQR) | 6039.2 (2875.2, 21027.0) | 4967.3 (2706.7, 10553.9) | 17866.9 (9822.0, 84821.3) |
| TG, mg/dL, mean ± SD | 297.1±134.5 | 277.9±138.3 | 309.5±147.2 |
| Fibrinogen, mg/dL, median (IQR) | 215.5 (154.8, 272.0) | 220.5 (184.8, 285.5) | 147.5 (86.3, 249.0) |
| Hemophagocytosis, n, (%) | 16 (80) | 12 (85.7) | 4 (66.7) |
| Hscore*, mean ± SD | 241.1 ± 42.9 | 237.9±38.8 | 248.5±54.7 |
| Underlying disease activity¥ | 20.2±7.9 | 7.5±1.4 |
¶ p-value < 0.05.
*HScore calculator (for the percentage probability of secondary HLH) is available at http://saintantoine.aphp.fr/score/.
¶¶Renal impairment was defined as an abrupt (within 48 hours) >50% decrease in eGFR or the need for renal replacement therapy (dialysis) during MAS treatment.
$eGFR is estimated using an equation developed by the Chronic Kidney Disease Epidemiology Collaboration.
¥SLEDAI-2K for SLE and Pouchot score for AOSD. MAS: Macrophage activation syndrome, HLH: Hemophagocytic lymphohistiocytosis, SLE: Systemic lupus erythematosus, AOSD: Adult-onset still’s disease, WBC: White blood cell, Hb: Hemoglobin, PLT: Platelet, AST: Aspartate aminotransferase, LDH: Lactate dehydrogenase, TB: Total bilirubin, eGFR, estimated glomerular filtration rate; ESR: Estimated sedimentation rate, CRP: C-reactive protein, TG: Triglyceride. SLEDAI-2K, Systemic Lupus Erythematosus Disease Activity Index-2000