Table 1.
Comparison between group 1 (j-NPSLE +) and group 2 (j-NPSLE– but j-SLE +)
| Parameters | Group 1, j-NPSLE + (n = 20) | Group 2, j-NPSLE − (n = 8) | P value |
|---|---|---|---|
| Age at j-SLE diagnosis (years), median (Q1; Q3) | 14.0 (11.3; 15.1) | 13.9 (12.2;14.6) | 0.950 |
| Sex ratio (female/male), n/n | 19/1 | 6/2 | 0.188 |
| Comorbiditiesa | |||
| Neuropsychiatric personal history, n (%)b | 5 (25) | 3 (38) | 0.651 |
| Familial history of auto-immune disease in a first degree relativec, n (%) | 3 (15) | 1 (13) | 1 |
| Laboratory findings at j-SLE diagnosis | |||
| Anti-nuclear antibody, n (%) | 20 (100) | 8 (100) | 1 |
| Anti-SSA, n (%) | 11 (58) | 3 (38) | 0.420 |
| Anti-SSB, n (%) | 0 | 1 (13) | 0.296 |
| Anti-Sm, n (%) | 13 (72) | 5 (63) | 0.667 |
| Anti-RNP, n (%) | 13 (68) | 4 (50) | 0.415 |
| Anti-ribosome P, n (%) | 5 (31) | 2 (33) | 1 |
| Anti-scl70, n (%) | 1 (6) | 1 (14) | 0.507 |
| Laboratory findings at NP evaluation | |||
| Anti-DNA, n (%) | 17 (85) | 7 (100) | 0.545 |
| Anemia (hemoglobin < 12.0 g/dL) | 17 (85) | 7 (88) | 1 |
| Leukopenia (white blood cells < 4 × 109/l) | 6 (30) | 4 (50) | 0.400 |
| Lymphopenia (absolute lymphocyte count < 1.5 × 109/L) | 14 (70) | 5 (63) | 1 |
| Neutropenia (absolute neutrophil count < 1.5 × 109/L) | 2 (10) | 2 (25) | 0.555 |
| Thrombocytopenia (< 150 × 109/l) | 7 (35) | 1 (13) | 0.372 |
| Erythrocyte sedimentation rate (mm), median (Q1; Q3) | 58 (49;105) | 43 (11;77) | 0.345 |
| C-reactive protein (mg/L), median (Q1; Q3) | 0 (0;14) | 0 (0;0) | 0.564 |
| Lupus anticoagulant, n (%) | 1 (6) | 0 | 1 |
| Anticardiolipin antibody, n (%) | 1 (6) | 3 (43) | 0.059 |
| Anti-beta2 glycoprotein antibody, n (%) | 1 (6) | 0 | 1 |
| Low complement (C3, C4, or CH50), n (%) | 17 (89) | 5 (63) | 0.136 |
| Clinical features at NP evaluation | |||
| SLEDAI#, median (Q1; Q3) | 23 (18; 27) | 8 (4; 15) | 0.096 |
| Cutaneous-mucosal, n (%) | 16 (80) | 4 (50) | 0.172 |
| Alopecia, n (%) | 6 (30) | 2 (25) | 1 |
| Cutaneous ulcerations, n (%) | 3 (15) | 0 | 0.536 |
| Chilblains, n (%) | 8 (40) | 2 (25) | 0.669 |
| Raynaud’s phenomenon, n (%) | 0 | 0 | |
| Rheumatological, n (%) | 12 (60) | 4 (50) | 0.691 |
| Hematological, n (%) | 16 (80) | 6 (75) | 1 |
| Renal, n (%) | 13 (65) | 2 (25) | 0.096 |
| Pericarditis, n (%) | 5 (25) | 0 | 0.281 |
| HTAP, n (%) | 1 (5) | 0 | 1 |
| Myocarditis, n (%) | 1 (5) | 0 | 1 |
| Pulmonary, n (%) | 8 (40) | 2 (25) | 0.669 |
| Pleurisy, n (%) | 4 (20) | 0 | 0.295 |
| Parenchymal, n (%) | 7 (35) | 2 (25) | 1 |
| Glaucome, n (%) | 2 (10) | 0 | 1 |
| Cumulative lupus clinical features | |||
| Cutaneous-mucosal, n (%) | 19 (95) | 8 (100) | 1 |
| Alopecia, n (%) | 13 (65) | 4 (50) | 0.672 |
| Cutaneous ulcerations, n (%) | 6 (30) | 2 (25) | 1 |
| Chilblains, n (%) | 12 (60) | 3 (38) | 0.410 |
| Raynaud’s phenomenon, n (%) | 2 (10) | 4 (50) | 0.038 |
| Rheumatological, n (%) | 17 (85) | 8 (100) | 0.536 |
| Hematological, n (%) | 19 (95) | 7 (88) | 0.497 |
| Renal, n (%) | 16 (80) | 6 (75) | 1 |
| Pericarditis, n (%) | 6 (30) | 1 (13) | 0.633 |
| HTAP, n (%) | 1 (5) | 0 | 1 |
| Myocarditis, n (%) | 1 (5) | 0 | 1 |
| Pulmonary, n (%) | 10 (50) | 3 (38) | 0.686 |
| Pleurisy, n (%) | 6 (30) | 2 (25) | 1 |
| Parenchymal, n (%) | 7 (35) | 3 (38) | 1 |
| Glaucoma, n (%) | 3 (15) | 0 | 0.536 |
| Treatments (cumulative) | |||
| Corticosteroid infusions, n (%) | 20 (100) | 6 (75) | 0.074 |
| Oral corticosteroids, n (%) | 20 (100) | 8 (100) | 1 |
| Cyclophosphamide, n (%) | 13 (65) | 1 (13) | 0.033 |
| Aspirin, n (%); curative anticoagulant, n (%) | 9 (45); 1 (5) | 3 (38); 2 (25) | 1; 0.188 |
| Hydroxychloroquine, n (%) | 19 (95) | 8 (100) | 1 |
| Mycophenolate mofetil or mycophenolic acid, n (%) | 18 (90) | 6 (75) | 0.555 |
| Anti-CD20: rituximab, n (%); obinutuzumab, n (%) | 4 (20); 2 (10) | 2 (25); 0 | 1; 1 |
| Methotrexate, n (%); azathioprine, n (%) | 1 (5); 2 (10) | 2 (25); 1 (13) | 0.188; 1 |
| Janus kinase inhibitors (ruxolitinib), n (%) | 1 (5) | 0 | 1 |
| Immunoadsorption, n (%); plasma exchanges, n (%) | 4 (20); 1 (5) | 0; 0 | 0.295; 1 |
| Tacrolimus, n (%) | 2 (10) | 0 | 1 |
| Abatacept, n (%); infliximab, n (%) | 1 (5); 0 | 0; 1 (13) | 1; 0.286 |
| Belimumab, n (%); eculizumab, n (%) | 0; 1 (5) | 1 (13); 0 | 0.286; 1 |
| Immunomodulatory IV immunoglobulins, n (%) | 1 (5) | 1 (13) | 0.497 |
| Outcome | |||
| J-SLE evolution from j-SLE diagnosis to last follow-up evaluation (months), median (Q1; Q3) | 27 (19;47) | 35 (29;48) | 0.609 |
| J-SLE evolution from NP evaluation to last follow-up evaluation (months), median (Q1; Q3) | 24 (16;37) | 14 (12;29) | 0.263 |
| Death, n (%) | 0 | 0 | |
| SLICC/ACR damage index score at last follow up: median (Q1; Q3); ≥ 1; n (%) | 1 (0;1); 11 (61) | 0 (0;0); 0 | 0.007; 0.007 |
IV intravenous, j-NPSLE juvenile neuropsychiatric systemic lupus erythematosus, j-SLE juvenile systemic lupus erythematosus, NP neuropsychiatric, SLEDAI# Systemic Lupus Erythematosus Disease Activity Index without neuropsychiatric features, SLICC systemic lupus international collaborating clinics, ACR American College of Rheumatology
aComorbidities in group 1: severe hypertriglyceridemia (1), immune thrombocytopenic purpura (1). In group 2: sickle cell disease (1), precocious puberty (1)
bNeuro-psychiatric personal history in group 1: Aicardi-Goutières syndrome with panic attack (1), learning disabilities (1), epilepsy (1), hemorrhagic stroke related to idiopathic thrombocytopenic purpura (1), nystagmus (1). In group 2: borderline personality disorder (2), transient ataxia (1)
cFamilial history of auto-immune disease in a first degree relative in group 1: SLE (1), Crohn’s disease (1), thyroiditis and dermatomyositis (1). In group 2: idiopathic thrombocytopenic purpura (1)