Characteristics | Group A(n = 8) | Group B (n = 49) | Group C (n = 39) | Significance |
---|---|---|---|---|
Mean onset age: years (range) | 6.8 (4.7-7.9) | 11(8.8-12.9) | 14.6(13–17.9) | |
Gender (F:M) | 7:1 | 2.5:1 | 4.6:1 | ns |
Positive family history (%) | 1(12.5) | 7 (14.3) | 7 (18) | ns |
Mean disease duration before diagnosis: months (range) | 21.3 (0.8-90) | 9.2 (0.1-96) | 4.4 (0.3-24) | ns |
Organ involvement (%) | ||||
Mucocutaneous | 6 (75) | 35 (71.4) | 29 (74.4) | ns |
Malar rash | 5 (62.5) | 26 (53.1) | 19 (48.7) | ns |
Discoid rash | 1 (12.5) | 3 (6.1) | 2 (5.1) | ns |
Oral ulcer | 4 (50) | 19 (38.8) | 12 (30.8) | ns |
Photosensitivity | 2 (25) | 6 (12.2) | 3 (7.7) | ns |
Alopecia | 0 (0) | 7 (14.3) | 7 (18) | ns |
Musculoskeletal | 5 (62.5) | 26 (53.1) | 21 (53.9) | ns |
Myalgia | 1 (12.5) | 3 (6.1) | 4 (10.3) | ns |
Arthritis | 4 (50) | 24 (49) | 19 (48.7) | ns |
Reticuloendothelial system | 3 (37.5) | 6 (12.2) | 6 (15.4) | ns |
Lymphadenopathy | 2 (25) | 4 (8.2) | 3 (7.7) | ns |
Splenomegaly | 1 (12.5) | 0 (0) | 2 (5.1) | ns |
Hepatic | 1 (12.5) | 2 (4.1) | 1 (2.6) | ns |
Gastrointestinal | 1 (12.5) | 2 (4.1) | 3 (7.7) | ns |
Renal | 1 (12.5) | 24 (49) | 23 (59) | p = 0.01 |
WHO class I | 0/0 (0) | 0/12 (0) | 0/8 (0) | ns |
WHO class II | 0/0 (0) | 1/12 (8.3) | 2/8 (25) | ns |
WHO class III | 0/0 (0) | 4/12 (33.3) | 0/8 (0) | ns |
WHO class IV | 0/0 (0) | 9/12 (75) | 5/8 (62.5) | ns |
WHO class V | 0/0 (0) | 4/12 (33.3) | 1/8 (12.5) | ns |
WHO class VI | 0/0 (0) | 0/12 (0) | 1/8 (12.5) | ns |
Central nervous system | 0 (0) | 3 (6.1) | 1 (2.6) | ns |
Serositis | 0 (0) | 5 (10.2) | 9 (23.1) | ns |
Pleural effusion | 0 (0) | 4 (8,2) | 6 (15.4) | ns |
Pericardial effusion | 0 (0) | 2 (4.1) | 5 (12.8) | ns |
Ascites/Peritonitis | 0 (0) | 2 (4.1) | 4 (10.3) | ns |
Hematologic | 6 (75) | 38 (77.6) | 34 (87.2) | ns |
Anemia | 6 (75) | 34 (69.4) | 30 (76.9) | ns |
Leukopenia | 0 (0) | 17 (34.7) | 13 (33.3) | ns |
Lymphopenia | 1 (12.5) | 18 (36.7) | 25 (64.1) | p = 0.005 |
Thrombocytopenia | 1 (12.5) | 9 (18.4) | 12 (30.8) | ns |
Laboratory (%) | ||||
Low C3 | 3 (37.5) | 41 (83.7) | 34 (87.2) | p = 0.004 |
Low C4 | 3 (37.5) | 42 (85.7) | 34 (87.2) | p = 0.03 |
Autoantibodies positive :number (%) | ||||
ANA | 8 (100) | 46 (93.9) | 36 (92.3) | ns |
Anti-dsDNA antibody | 5 (62.5) | 44 (89.8) | 30 (76.9) | ns |
aCL | 0/2 (0) | 4/12 (33.3) | 3/18 (16.7) | ns |
LAC | 0/2 (0) | 1/13 (7.7) | 1/18 (5.6) | ns |
Anti-ENA | ||||
Anti-RNP | 3/6 (50) | 10/30 (33.3) | 4/29 (13.8) | ns |
Anti-Jo-1 | 1/6 (16.7) | 0/30 (0) | 0/29 (0) | p = 0.012 |
Anti-Scl-70 | 0/6 (0) | 3/30 (10) | 1/29 (3.4) | ns |
Anti-Ro | 4/6 (66.6) | 10/30 (33.3) | 11/29 (37.9) | ns |
Anti-La | 2/6 (33.3) | 4/30 (13.3) | 3/29(10.3) | ns |
Anti-Sm | 1/6 (16.7) | 10/30 (33.3) | 0/29 (0) | p = 0.009 |