Characteristics of 355 patients with ANA-associated AIC
| . | Patients with ANA, n = 355 . | Patients with ANA and SLE, n = 79 . | Patients with ANA but negative for SLE, n = 276 . | P value . |
|---|---|---|---|---|
| Sex ratio (female/male) | 252/103 | 72/7 | 180/96 | 2.3e-6 |
| Median age at AIC diagnosis, y (min-max) | 10.5 (0.2-17.6) | 12.4 (0.8-17.0) | 9.7 (0.2-17.6) | 4.7e-5 |
| AIC type at initial diagnosis, n (%) cITP | 247 (70%) | 49 (62%) | 198 (72%) | .13 |
| isolated AIHA | 70 (20%) | 13 (16%) | 57 (21%) | .52 |
| simultaneous ES | 38 (10%) | 17 (22%) | 21 (8%) | .001 |
| Severity of AIC at initial diagnosis, n (%) | ||||
| Clinical severity, n (%) | 69/214 (32%) | 24/52(46%) | 45/162 (28%) | .02 |
| Hematological severity, n (%) | 138/340 (41%) | 38/77 (49%) | 100/263 (38%) | .08 |
| First-degree or second-degree familial IM, n (%) | 131/355 (37%) | 35/79 (44%)∗ | 96/276 (35%) | .14 |
| cIM other than SLE during the follow-up, n (%) | 62/355 (17%) | 15/79 (19%)† | 51/276 (18%) | 1 |
| ANA titer during follow-up ≥1/320, n (%) | 225/355 (64%) | 65/79 (82%) | 160/276 (58%) | 6e-5 |
| Patients with second-line treatments, n (%) | 238/355 (67%) | 72/79 (91%) | 166/276 (60%) | 5.9e-8 |
| Hydroxychloroquine, n (%) | 143 (40%) | 65 (82%) | 78 (28%) | 4.7e-18 |
| IS (AZA, MMF, CYC, CSA, VCR), n (%) | 104 (29%) | 31 (39%) | 73 (26%) | .04 |
| Rituximab, n (%) | 93 (26%) | 23 (29%) | 70 (25%) | .56 |
| Splenectomy, n (%) | 38 (11%) | 11 (14%) | 27 (10%) | .30 |
| Patients died, n (%) | 7 (2%) | 2 (3%) | 5 (1%) | .65 |
| Patients in CR at 5 years from initial AIC diagnosis, n (%) | 107/207 (51%) | 32/51 (63%) | 75/156 (46%) | .07 |
| Median follow-up from AIC diagnosis, y (min-max) | 5.8 (0.1-29.6) | 5.4 (0.2-20.1) | 5.8 (0.1-29.6) | .99 |
| . | Patients with ANA, n = 355 . | Patients with ANA and SLE, n = 79 . | Patients with ANA but negative for SLE, n = 276 . | P value . |
|---|---|---|---|---|
| Sex ratio (female/male) | 252/103 | 72/7 | 180/96 | 2.3e-6 |
| Median age at AIC diagnosis, y (min-max) | 10.5 (0.2-17.6) | 12.4 (0.8-17.0) | 9.7 (0.2-17.6) | 4.7e-5 |
| AIC type at initial diagnosis, n (%) cITP | 247 (70%) | 49 (62%) | 198 (72%) | .13 |
| isolated AIHA | 70 (20%) | 13 (16%) | 57 (21%) | .52 |
| simultaneous ES | 38 (10%) | 17 (22%) | 21 (8%) | .001 |
| Severity of AIC at initial diagnosis, n (%) | ||||
| Clinical severity, n (%) | 69/214 (32%) | 24/52(46%) | 45/162 (28%) | .02 |
| Hematological severity, n (%) | 138/340 (41%) | 38/77 (49%) | 100/263 (38%) | .08 |
| First-degree or second-degree familial IM, n (%) | 131/355 (37%) | 35/79 (44%)∗ | 96/276 (35%) | .14 |
| cIM other than SLE during the follow-up, n (%) | 62/355 (17%) | 15/79 (19%)† | 51/276 (18%) | 1 |
| ANA titer during follow-up ≥1/320, n (%) | 225/355 (64%) | 65/79 (82%) | 160/276 (58%) | 6e-5 |
| Patients with second-line treatments, n (%) | 238/355 (67%) | 72/79 (91%) | 166/276 (60%) | 5.9e-8 |
| Hydroxychloroquine, n (%) | 143 (40%) | 65 (82%) | 78 (28%) | 4.7e-18 |
| IS (AZA, MMF, CYC, CSA, VCR), n (%) | 104 (29%) | 31 (39%) | 73 (26%) | .04 |
| Rituximab, n (%) | 93 (26%) | 23 (29%) | 70 (25%) | .56 |
| Splenectomy, n (%) | 38 (11%) | 11 (14%) | 27 (10%) | .30 |
| Patients died, n (%) | 7 (2%) | 2 (3%) | 5 (1%) | .65 |
| Patients in CR at 5 years from initial AIC diagnosis, n (%) | 107/207 (51%) | 32/51 (63%) | 75/156 (46%) | .07 |
| Median follow-up from AIC diagnosis, y (min-max) | 5.8 (0.1-29.6) | 5.4 (0.2-20.1) | 5.8 (0.1-29.6) | .99 |
AZA, azathioprine; cIM, clinical immune manifestation; CR, complete remission; CSA, cyclosporin A; CYC, cyclophosphamide; IM, immunopathological manifestations; IS, immunosuppressants; MMF, mycophenolate mofetil; VCR, vincristine. Boldfaced P values indicate P value > .05.
Main significant familial history, that is, first- or second-degree relatives with IM for patients with ANA who had SLE: cancer (n = 10), SLE (n = 8), thyroid disease (n = 7), arthritis (n = 5), inflammatory bowel disease (n = 4), myasthenia (n = 2), celiac disease (n = 2), and type 1 diabetes (n = 2).
Clinical IM observed for patients with ANA who had SLE: autoimmune thyroiditis (n = 8), acrosyndrome (n = 2), cryoglobulinemia type IIa (n = 1), inflammatory bowel disease (n = 1), neuromyelitis optica associated with antiaquaporin 4 antibodies (n = 1), pernicious anemia (n = 1), and rheumatoid arthritis (n = 1).