Table 1.
Demographic, clinical and laboratory characteristics of incident patients with childhood-onset systemic lupus erythematosus from January 1, 2000 to June 30, 2016.
| All cSLE (n=130) N (%) | cSLE with AC (n=43 ) N (%) | cSLE without AC (n=87) N (%) | P-value | Missing | |
|---|---|---|---|---|---|
| Demographics | |||||
| Sex | |||||
| Female | 107 (82) | 35/43 (81) | 72/87 (83) | 0.848 | |
| Race | 0.620 | 8 | |||
| Black | 81 (66) | 26/41 (63) | 55/81 (68) | ||
| Other | 41 (34) | 15/41 (37) | 26/81 (32) | ||
| Age in years at cSLE diagnosis (mean (SD))c | 12 (3) | 13 (3) | 12 (3) | 0.321 | |
| Clinical features | |||||
| Fever | 54 (42) | 21/42 (50) | 33/87 (38) | 0.193 | 1 |
| Malar rash | 47 (36) | 10/42 (24) | 37/87 (43) | 0.038S | 1 |
| Photosensitivity | 47 (36) | 13/42 (33) | 34/87 (39) | 0.369 | 1 |
| Discoid lupusa | 3 (2) | 1/42 (2) | 2/87 (2) | 1.000 | 1 |
| Vasculitic rash | 33 (26) | 13/42 (31) | 20/87 (23) | 0.331 | 1 |
| Raynaudsa | 14 (11) | 5/42 (12) | 9/87 (10) | 0.770 | 1 |
| Oral ulcers | 31 (24) | 9/42 (21) | 22/87 (25) | 0.631 | 1 |
| Nasal ulcersa | 4 (3) | 2/42 (5) | 2/87 (2) | 0.596 | 1 |
| Alopecia | 21 (16) | 4/42 (10) | 17/87 (20) | 0.149 | 1 |
| Arthritis | 64 (50) | 13/42 (31) | 51/86 (60) | 0.003S | 2 |
| Angioedemaa | 8 (6) | 2/41 (5) | 6/86 (7) | 1.000 | 3 |
| Pleural effusion | 26 (31) | 12/32 (38) | 14/53 (26) | 0.283 | 45 |
| Pericardial effusion | 17 (32) | 7/19 (37) | 10/34 (29) | 0.578 | 77 |
| Neuropsychiatric symptomsa | 8 (6) | 6/43 (14) | 2/87 (2) | 0.016S | |
| Myositis* | 20 (29) | 2/20 (10) | 18/49 (37) | 0.026S | 61 |
| Laboratory features | |||||
| Positive ANA ≥ 1:40 | 130 (100) | 43 (100) | 87 (100) | ||
| Positive anti-dsDNA ≥ 1:10 | 88 (70) | 31/43 (72) | 57/83 (69) | 0.692 | 4 |
| Positive anti-RNP* | 72 (58) | 24/43 (56) | 48/81 (59) | 0.711 | 6 |
| Positive anti-Smith* | 71 (57) | 22/43 (51) | 49/81 (61) | 0.318 | 6 |
| Positive anti-SSA* | 56 (45) | 20/43 (47) | 36/81 (44) | 0.826 | 6 |
| Positive anti-SSB* | 20 (16) | 10/43 (23) | 10/80 (13) | 0.123 | 7 |
| Leukopenia ≤ 4,000/uL | 59 (46) | 21/43 (49) | 38/85 (45) | 0.658 | 2 |
| Lymphopenia ≤1,500/uL | 81 (66) | 29/42 (69) | 52/81 (64) | 0.591 | 7 |
| Neutropenia ≤1,500/uL | 32 (27) | 14/42 (33) | 18/79 (23) | 0.210 | 9 |
| Low C3 complement* | 63 (53) | 25/41 (61) | 38/79 (48) | 0.180 | 10 |
| Low C4 complement* | 78 (65) | 29/41 (71) | 49/79 (62) | 0.343 | 10 |
| ESR in mm/hr (mean(SD)) | 63 (41) | 80 (45) | 55 (36) | 0.003S | 19 |
| eGFR < 90mL/min/1.73m2 | 32 (29) | 14/39 (36) | 18/73 (25) | 0.210 | 18 |
| SLEDAI-2k (median(IQR,range) | 9 (7 – 12) | 9 (6 – 11) | 10 (7 – 12) | 0.655 | 11 |
| Prior Treatment | |||||
| Corticosteroidsa | 10 (8) | 10/43 (23) | 0/87 (0) | <0.001S | |
| Cyclophosphamide | 0 (0) | 0/43 (0) | 0/87 (0) | ||
| IVIGa | 7 (5) | 7/43 (16) | 0/87 (0) | <0.001S | |
| Rituximaba | 2 (2) | 2/43 (5) | 0/87 (0) | 0.108 | |
| 2 year risk of lupus nephritis after cSLE diagnosis | 16 (12) | 3/43 (7) | 13/87 (15) | 0.190 | |
| Follow-up period in years (mean(SD)) | 4.03 (2) | 3.96 (2) | 4.16 (2) | 0.641 |
Abbreviations: AC = Autoimmune cytopenias; cSLE = childhood-onset systemic lupus erythematosus; dsDNA = double-stranded DNA; ESR = Erythrocyte sedimentation rate; eGFR = Estimated glomerular filtration rate; IVIG = intravenous immunoglobulin; RNP = ribonucleoprotein; SLEDAI-2k = Systemic Lupus Erythematosus Disease Activity Index 2000; SS = Sjogren syndrome-related antigen
S
= significant with p value < 0.05
*
By laboratory reference range, Comparisons were by chi square test except otherwise stated
a
Fisher’s exact test
b
Wilcoxon rank sum test
c
Student’s t-test