Characteristics of the patients with ANCA-associated vasculitis who were included in this study*
| All patients (n=323) | No malignancy occurrence (n=290) | Malignancy occurrence (n=33) | p Value† | |
|---|---|---|---|---|
| Age (years) at diagnosis, mean (SD) | 56.4 (16.1) | 55.9 (16.3) | 61.3 (12.7) | 0.03 |
| Follow-up (years), mean (SD) | 5.6 (3.2) | 5.5 (3.2) | 6.3 (3.2) | 0.20 |
| Male, n (%) | 149 (46) | 135 (47) | 14 (42) | 0.65 |
| Clinical diagnosis, n (%) | 0.64 | |||
| Microscopic polyangiitis | 160 (49) | 146 (50) | 14 (42) | |
| Granulomatosis with polyangiitis | 109 (34) | 97 (33) | 12 (36) | |
| Eosinophilic granulomatosis with polyangiitis | 54 (17) | 47 (16) | 7 (21) | |
| ANCA serotype, n (%)‡ | 0.89 | |||
| MPO-ANCA | 110 (34) | 99 (34) | 11 (33) | |
| PR3-ANCA | 152 (47) | 136 (47) | 16 (49) | |
| Organ involvement, mean (SD) | 2.3 (1.5) | 2.3 (1.5) | 2.2 (1.2) | 0.85 |
| Deaths, n (%) | 39 (12) | 30 (10) | 9 (27) | 0.01 |
| Relapsing disease, n (%) | 86 (28) | 79 (28) | 7 (22) | 0.54 |
| Renal transplantation, n (%) | 12 (4) | 11 (4) | 1 (3) | 1.00 |
| Treatment, n (%) | ||||
| Glucocorticoids | 318 (99) | 286 (99) | 32 (97) | 0.33 |
| Cyclophosphamide | 233 (72) | 207 (72) | 26 (79) | 0.38 |
| Rituximab | 155 (48) | 144 (50) | 11 (33) | 0.07 |
| Cyclophosphamide and rituximab | 114 (35) | 105 (36) | 9 (27) | 0.31 |
| Azathioprine | 218 (68) | 196 (68) | 22 (67) | 0.89 |
| Mycophenolate mofetil | 154 (48) | 141 (50) | 13 (39) | 0.31 |
| Methotrexate | 39 (12) | 35 (12) | 4 (12) | 1.00 |
| TNF-α inhibitors | 19 (6) | 15 (5) | 4 (12)§ | 0.12 |
*Values are reported as means (SD) or as numbers (%).
†p Values were calculated using Student's t-test, χ2 test or Fisher's exact test.
‡ANCA serotype data were not available for 61 patients.
§Four of the 19 patients (21%) who received TNF-α inhibitors developed, in total, two basal cell carcinomas, one breast carcinoma and one prostate carcinoma. All four patients were also treated with cyclophosphamide, and one was treated with rituximab. Malignancy risk was similar in patients treated with and without a TNF-α inhibitor.
ANCA, antineutrophil cytoplasmic antibody; MPO-ANCA, myeloperoxidase-ANCA; PR3-ANCA, proteinase 3 ANCA; TNF, tumour necrosis factor.