Table 2.
Type of neutropenic episodes and associations between confirmed neutropenia and disease activity, fulfilled classification criteria, presence of autoantibodies, and ongoing treatment
| Episodes (n = 94) | Percent of all episodes (%) | Patients (n = 40) | |
|---|---|---|---|
| Occasional neutropenia (1 episode only) | 24 | 26 | 24 |
| Recurrent neutropenia (> 1 episode) | 70 | 75 | 16 |
| Mild neutropenia | 62 | 66 | 36 |
| Moderate neutropenia | 21 | 22 | 8 |
| Severe neutropenia | 9 | 9.6 | 1 |
| Agranulocytosis | 2 | 2.1 | 2 |
| Neutropenia with clinical SLEDA-2 K score ≤ 4 | 76 | 81 | 33 |
| Neutropenia with ≥ 4 ACR criteria fulfilled | 84 | 89 | 38 |
| Neutropenia with Renal disorder (7th ACR criterion fulfilled) | 16 | 17 | 12 |
| Neutropenia with ≥ 1 concomitant hematologic manifestation | 88 | 94 | 34 |
| Neutropenia with positivity for anti-SSA antibodies (Ro60, Ro52/TRIM21) | 58 | 62 | 27 |
| Neutropenia with positivity for anti-SSB antibodies (La) | 26 | 28 | 14 |
| Neutropenia with Immunologic disorder (10th ACR criterion fulfilled) | 39 | 42 | 25 |
| Neutropenic episodes with ongoing daily treatment with 0–5 mg prednisolone | 58 | 62 | 29 |
| Neutropenic episodes with ongoing daily treatment with > 5 mg prednisolone | 36 | 38 | 15 |
| Neutropenic episodes with ongoing treatment with ≥ 1 immunosuppressive agent* | 31 | 33 | 18 |
| Neutropenic episodes with ongoing treatment with rituximab | 8 | 9 | 6 |
| Neutropenic episodes with ongoing treatment with belimumab | 1 | 1 | 1 |
*Immunosuppressive agents provided: sirolimus (n = 11), cyclosporine (n = 5), methotrexate (n = 5), azathioprine (n = 4), mycophenolate mofetil (n = 4), leflunomide (n = 2), dapsone (n = 1), and cyclophosphamide (n = 1)