Table 1.
Characteristics of 77 paediatric onset LS patients who were followed clinically for 2 years or more, grouped by those that had a relapse of disease and those that did not
| Characteristics | Total (n=77) |
Relapse (n=35) |
No Relapse (n=42) |
p-value1 |
|---|---|---|---|---|
| Gender | n (%) | n (%) | n (%) | |
| Female | 54 (70) | 26 (74) | 28 (62) | 0.23 |
| LS Subtype | ||||
| Circumscribed Superficial | 21 (27) | 10 (29) | 11 (26) | 1 |
| Circumscribed Deep | 8 (10) | 4 (11) | 4 (10) | 1 |
| Generalized Morphea | 10 (13) | 6 (17) | 4 (10) | 0.5 |
| Linear Trunk/Limb | 42 (55) | 20 (57) | 22 (52) | 0.81 |
| Linear Face | 12 (16) | 3 (9) | 9 (21) | 0.21 |
| Mixed Morphea | 12 (16) | 6 (17) | 6 (14) | 0.76 |
| Pansclerotic Morphea | 2 (2.6) | --- | 2 (4.5) | --- |
| Eosinophilic Fasciitis | 1 (1.3) | --- | 1 (2.4) | --- |
| Laboratory Evaluation* | ||||
| ANA Positive‡ | 26/63 (41) | 18/30 (60) | 8/33 (24) | <0.01 |
| ssDNA Positive | 31/71 (44) | 13/32 (41) | 18/39 (46) | 0.81 |
| AHA Positive | 26/70 (37) | 9/31 (29) | 17/39 (44) | 0.23 |
| CPK Elevated | 18/71 (25) | 10/34 (29) | 8/37 (22) | 0.59 |
| Aldolase Elevated | 22/66 (33) | 13/32 (41) | 9/34 (27) | 0.21 |
| Extracutaneous Manifestations | ||||
| At least one ECM | 33 (43) | 10 (28) | 23 (55) | 0.02 |
| Joint Contractures | 23 (30) | 8 (23) | 15 (35) | 0.22 |
| Arthritis | 3 (3.9) | 2 (5.7) | 1 (2.4) | 0.59 |
| Dental | 4 (5.2) | --- | 4 (9.5) | --- |
| Uveitis | 1 (1.2) | --- | 1 (2.4) | --- |
| Limb length discrepancy | 5 (6.5) | 1 (2.9) | 4 (9.5) | 0.37 |
| Limb circumference difference | 14 (18) | 6 (17) | 8 (19) | 0.047 |
| Medication | ||||
| On MTX and CS regimen¥ | 67 (87) | 28 (80) | 39 (93) | 0.09 |
| Compliance with medication | 18 (23) | 11 (31) | 7 (17) | 0.13 |
| mean (SD) | mean (SD) | p-value | ||
| Age Onset (years) | 10.0 (3.9) | 6.7 (3.7) | <0.001 | |
| Time Onset to Diagnosis (mos) | 13.8 (18.5) | 22.4 (31.4) | 0.16 | |
| Follow-up Duration (years) | 4.3 (2.0) | 4.9 (2.8) | 0.34 | |
P-values were obtained using Chi-squared tests for categorical variables and Fisher exact tests when n<5 for a cell. Independent sample t-tests were used for continuous variables. Comparisons were made between relapse and non-relapse groups.
Laboratory parameters not obtained for all patients so denominators are provided.
ANA (antinuclear antibody), ssDNA (single stranded DNA antibody), AHA (antihistone antibody), CPK (creatine phosphokinase)
Patients were routinely treated according to consensus protocols with a standardized regimen of subcutaneous (SC) MTX at 1 mg/kg/week (maximum 25mg/week) and oral prednisone 2mg/kg/day (maximum 60mg/day). MTX SC was continued for 24 months and then switched to oral administration to complete 36 months of therapy. Prednisone was tapered and kept at 0.25mg/kg/day for 12 months (Ref 2, 8)
The ANA titer and pattern was varied among patients in both relapse and nonrelapse groups, range 1:80 to 1:640