Table 2.
Treatment of AAHS in adults*
| Total (n = 116)† | Underlying SLE (n = 61) | Underlying AOSD (n = 31) | |
|---|---|---|---|
| Treatment, no. (%)‡ | |||
| Corticosteroids | 114 (98.3) | 61 (100) | 30 (96.8) |
| Prednisolone | 79 (68.1) | 50 (82.0) | 21 (70.0) |
| IV MP | 71 (61.2) | 38 (62.3) | 17 (56.7) |
| IVIG | 28 (24.1) | 12 (19.7) | 8 (25.8) |
| Cyclosporine | 24 (20.7) | 13 (21.3) | 8 (25.8) |
| IV CYC | 17 (14.7) | 11 (18.0) | 3 (9.7) |
| G-CSF | 8 (6.9) | 2 (3.3) | 2 (6.5) |
| Plasma exchange | 5 (4.3) | 3 (4.9) | 2 (6.5) |
| Tacrolimus | 5 (4.3) | 2 (3.3) | 1 (3.2) |
| Methotrexate | 4 (3.4) | 1 (1.6) | 3 (9.7) |
| Etoposide | 3 (2.6) | 1 (1.6) | 1 (3.2) |
| Vincristine | 3 (2.6) | 1 (1.6) | 1 (3.2) |
| Splenectomy | 2 (1.7) | 1 (1.6) | 0 (0) |
| Leukapheresis | 1 (0.9) | 0 (0) | 0 (0) |
| CHOP | 1 (0.9) | 0 (0) | 1 (3.2) |
| Biologic agents | |||
| Infliximab | 2 (1.7) | 1 (1.6) | 1 (3.2) |
| Etanercept | 3 (2.6) | 2 (3.3) | 1 (3.2) |
| Rituximab | 3 (2.6) | 3 (4.9) | 0 (0) |
| Tocilizumab | 1 (0.9) | 0 (0) | 1 (3.2) |
| Effects of treatment, no. of responders/total no. (%) | |||
| Initial therapy | |||
| Corticosteroids | 64/111 (57.7) | 32/59 (54.2) | 18/30 (60) |
| Corticosteroids alone | 46/87 (52.9) | 28/53 (52.8) | 11/20 (55) |
| Corticosteroids + other agents§ | 18/24 (75)¶ | 4/6 (66.7) | 7/10 (70) |
| IVIG | 1/4 (25) | 0/2 (0) | 1/1 (100) |
| Cyclosporine | 1/1 (100) | ||
| Overall response to initial therapy | 66/116 (56.9) | 32/61 (52.4) | 19/31 (61.3) |
| Therapy for corticosteroid-refractory disease# | |||
| Cyclosporine | 5/14 (35.7) | 2/10 (20) | 2/3 (66.7) |
| IV CYC | 11/12 (91.6)** | 5/6 (83.3)** | 5/5 (100) |
| IVIG | 1/12 (8.3) | 0/7 (0) | 0/1 (0) |
Dosages of cyclosporine ranged from 2 mg/kg/day to 5 mg/kg/day, and dosages of intravenous cyclophosphamide (IV CYC) ranged from 0.5 gm to 1 gm per pulse. IV MP = IV methylprednisolone; G-CSF = granulocyte colony-stimulating factor; CHOP = cyclophosphamide, doxorubicin, vincristine, and prednisone.
Includes the 61 patients with underlying systemic lupus erythematosus (SLE), the 31 with underlying adult-onset Still's disease (AOSD), and the 24 with other autoimmune diseases underlying autoimmune-associated hemophagocytic syndrome (AAHS).
Includes initial therapy and therapy for refractory disease.
Includes cyclosporine, tacrolimus, IV CYC, or methotrexate.
P < 0.05 versus corticosteroids alone.
The most frequently used treatments were evaluated.
P < 0.01 versus cyclosporine or IV immunoglobulin (IVIG).