Clinical features of 18 patients with macrophage activation syndrome (MAS).
| Patient | Age/Sex | Underlying Disease and Duration (m/*) | Organ Involvement | HPS | Treatment | Outcome |
|---|---|---|---|---|---|---|
| 1 | 29 F | SLE (96/25) | Alveolar hemorrhage, pleural effusion, pericardial effusion, DIC | — | Pulsed mPSL + PSL (1.2 mg/kg) | Died |
| 2 | 26 F | SLE (12/32) | GI bleeding, alveolar hemorrhage, PH, DIC, nephritis, blurred vision, altered mental status | NA | Pulsed mPSL + IVIg + CyA (3 mg/kg) | Died |
| 3 | 18 F | SLE (4/56) | Cerebritis, nephritis, enteritis, pleural effusion, pericardial effusion | + | Pulsed mPSL | Died |
| 4 | 32 F | SLE (12/54) | Interstitial pneumonia | — | Pulsed mPSL + PSL (1.2 mg/kg) → IVIg → CyA (5 mg/kg) | Recovered |
| 5 | 76 F | SLE (7/18) | Interstitial pneumonia, pleural effusion, pericardial effusion | — | PSL (10 mg/d) | Recovered |
| 6 | 35 F | SLE (24/36) | Pleural effusion | NA | Pulsed mPSL + PSL (1.2 mg/kg) | Recovered |
| 7 | 14 M | SLE (0/9) | — | + | Pulsed mPSL + PSL (1.2 mg/kg) | Recovered |
| 8 | 64 F | SLE (258/21) | Pleural effusion | — | Pulsed mPSL + PSL (5 mg/d) → IVIg | Recovered |
| 9 | 64 F | SLE (432/21) | PH, pleural effusion, ascitis, renal failure, heart failure | — | PSL (1.2 mg/kg) | Recovered† |
| 10 | 70 F | AOSD (3) | Interstitial pneumonia | + | Pulsed mPSL + PSL (1.2 mg/kg) + IVIg | Recovered†† |
| 11 | 16 F | AOSD (0) | Altered mental status | — | PSL (1.2 mg/kg) → IVIg → pulsed mPSL | Recovered |
| 12 | 36 F | AOSD (3) | ARDS, peripheral neuropathy | + | Pulsed mPSL + PSL (1.2 mg/kg) | Recovered |
| 13 | 30 F | AOSD (148) | Pleural effusion, pericardial effusion | NA | PSL (26 mg/d) + CyA (2.5 mg/kg) → pulsed mPSL | Recovered |
| 14 | 36 F | AOSD (8) | Pleural effusion, nephritis, uveitis | NA | IPF (30 mg/kg) → PSL (1.2 mg/kg) → pulsed mPSL | Recovered |
| 15 | 76 F | AOSD (1) | Pleural effusion | NA | IPF (30 mg/kg) → PSL (0.8 mg/kg) → pulsed mPSL → IVIg → CyA (3 mg/kg) | Recovered |
| 16 | 31 F | AOSD (45) | Nephritis, colitis | NA | PSL (0.8 mg/kg for 3 days) → PSL (10 mg/d) | Recovered |
| 17 | 52 F | RA (31) | ARDS, DIC, GI bleeding | NA | Pulsed mPSL | Died |
| 18 | 55 F | APS (396) | GGO | NA | PSL (5 mg/d) + warfarin (2.5 mg/d) | Recovered |
m: month;
↵* Systemic Lupus Erythematosus Disease Activity Index (SLEDAI); HPS: hemophagocytosis; AOSD: adult-onset Still’s disease; RA: rheumatoid arthritis; APS: antiphospholipid syndrome; DIC: disseminated intravascular coagulopathy; GI: gastrointestinal; PH: pulmonary hypertension; ARDS: acute respiratory distress syndrome; GGO: ground-glass opacity in chest computed tomography; NA: not available; mPSL: methlyprednisolone; PSL: prednisolone; IVIg: intravenous immunoglobulin; CyA: cyclosporin A; IPF: ibuprofen.
↵† Died of cytomegalovirus infection 1 month after recovery from MAS.
↵†† Died of severe pneumonia complicated with tension pneumothorax 1.5 months after recovery from MAS.