Table 1.

Systemic lupus erythematosus features at diagnosis.

Cohort 1, n = 34Cohort 2, n = 41
MAS, (n = 10) (%)Non-MAS (n = 24) (%)MAS (n = 10) (%)Non-MAS (n = 31) (%)
Demographics
Age at diagnosis, yrs, mean ± SD (range)14.9 ± 1.513.6 ± 2.813.6 ± 2.913.6 ± 2.4
(11.9–16.5)(9–17.1)(7.8–16.6)(8.8–17.2)
Female8 (80)21 (88)8 (80)25 (81)
Organ system involvement
Arthritis5 (50)19 (79)8 (80)18 (58)
Mucocutaneous involvement
    Malar rash8 (80)8 (33)5 (50)24 (77)
    Other rash5 (50)11 (46)3 (30)7 (22)
    Oral ulcers5 (50)8 (33)3 (30)8 (25)
    Alopecia3 (30)14 (58)2 (20)6 (19)
    Photosensitivity2 (20)6 (25)2 (20)5 (16)
    Nasal ulcers1 (10)4 (17)2 (20)3 (9)
    Digital ulcers01 (4)2 (20)2 (6)
Lupus nephritis (any)3 (30)11 (46)5 (50)14 (44)
    Mesangial (class II)01 (4)2 (20)1 (3)
    Focal proliferative (class III)1 (10)4 (17)2 (20)5 (16)
    Diffuse proliferative (class IV)1 (10)4 (17)1 (10)6 (19)
    Membranous (class V)1 (10)4 (17)03 (9)
    Nephrotic syndrome1 (10)5 (21)05 (16)
Central nervous system (any)2 (20)10 (42)1 (10)7 (23)
        Psychosis03 (13)1 (10)1 (3)
        Cerebrovascular disease2 (20)5 (21)01(3)
        Cognitive dysfunction06 (25)1 (10)2 (6)
Pericarditis06 (25)1 (10)9 (28)
Pleuritis1 (10)5 (21)3 (30)9 (29)
Myositis02 (8)02 (6)
Diffuse lymphadenopathy6 (60)8 (33)2 (20)7 (22)
Raynaud phenomenon1 (10)2 (8)02 (6)
Fatigue9 (90)17 (71)9 (90)17 (53)
Fever10 (100)24 (100)10 (10)32 (100)
Weight loss4 (40)14 (58)4 (4)16 (50)
Anorexia5 (50)9 (38)6 (6)11 (34)
Headache3 (30)6 (25)1 (10)6 (19)
Autoantibodies
ANA10 (100)24 (100)10 (100)31 (100)
Anti-dsDNA8 (80)21 (88)9 (90)25 (81)
Anti-Sm6 (60)11 (46)4 (40)13 (41)
Anti-RNP7 (70)12 (50)4 (40)28 (90)
Anti-Ro4 (40)12 (50)5 (50)15 (47)
Anti-La1 (10)3 (13)2 (20)4 (13)
Antiphospholipid (any)3 (30)19 (79)5 (50)9 (28)
    Anticardiolipin3 (30)19 (79)4 (40)7 (22)
    LAC1 (10)3 (13)1 (10)4 (13)
    Rheumatoid factor02 (8)00
Hematologic
Thrombocytopenia6 (60)7 (29)7 (70)7 (23)
Lymphopenia9 (90)15 (63)9 (90)21 (68)
Coombs-positive hemolytic anemia2 (20)10 (42)7 (70)16 (52)
Positive DAT7 (70)18 (75)8 (80)19 (59)
Leukopenia1 (10)02 (20)7 (23)
Neutropenia001 (10)1 (3)
  • Values are expressed as n (%) unless otherwise indicated. ANA: antinuclear antibody; DAT: direct antiglobulin test; LAC: lupus anticoagulant; MAS: macrophage activation syndrome.