Characteristics | All Patients | CTP A (MTX) | CTP B (MTX + IV CS) | CTP C (MTX + PO CS) | p |
---|---|---|---|---|---|
No. patients | 44 | 12 (27.3) | 23 (52.3) | 9 (20.5) | NS |
Age at study entry, yrs, median (IQR) | 12.6 (9.6–14.3) | 11.8 (9.8–14.1) | 11.6 (6.5–15.1) | 13.3 (12.2–14.2) | NS |
Age of onset, yrs, median (IQR) | 9.4 (5.9–11.8) | 9.3 (6.8–11.5) | 9.7 (4.5–10.6) | 10.5 (5.7–13.4) | NS |
Disease duration, mos, median (IQR) | 12.5 (6–57.5) | 19 (6.5–38.5) | 13 (6–72) | 10 (6–65) | NS |
Female | 31 (70.5) | 10 (83) | 15 (65) | 6 (67) | NS |
Race | NS | ||||
White | 41 (93.2) | 10 (83) | 22 (96) | 9 (100) | |
Asian | 2 (4.6) | 2 (17) | 0 | 0 | |
Mixed | 1 (2.3) | 0 | 1 (4) | 0 | |
Hispanic | 9 (20.5) | 3 (25) | 4 (17) | 2 (22) | NS |
Subtype1 | 0.088 | ||||
Circumscribed superficial | 3 (6.8) | 0 | 1 (4) | 2 (22) | |
Circumscribed deep | 2 (4.6) | 0 | 0 | 2 (22) | |
Linear scleroderma | 28 (63.6) | 9 (75) | 14 (61) | 5 (56) | |
Generalized morphea | 1 (2.3) | 0 | 1 (4) | 0 | |
Pansclerotic morphea | 1 (2.3) | 0 | 1 (4) | 0 | |
Mixed morphea | 9 (20.5) | 3 (25) | 6 (26) | 0 | |
Affected anatomic sites, median (IQR) | 2 (1.5–4) | 2 (1.5–4) | 3 (2–5) | 2 (1–3) | 0.021 |
Extracutaneous involvement | 33 (75) | 10 (83) | 16 (69.6) | 7 (77.8) | NS |
Growth disturbance1 | 21 (47.7) | 7 (58.3) | 13 (56.5) | 1 (11) | 0.062 |
Joint involvement3 | 20 (45.5) | 6 (50) | 13 (56.5) | 1 (11) | NS |
Prior systemic treatment | 7 (15.9) | 1 (8) | 6 (26) | 0 | NS |
ANA positivity (among those evaluated) | 23 (60.5) | 5 (50) | 15 (79) | 3 (33) | 0.083 |
Elevated ESR/CRP (among those evaluated)2 | 10 (25.0) | 0 | 8 (36) | 2 (25) | 0.090 |
Family history of an autoimmune disease | 21 (48.8) | 6 (50) | 10 (45.5) | 5 (56) | NS |
Values are n (%) unless otherwise specified. P values for differences across the 3 CTP are shown in last column. Significant p values are in bold face; p values that approached significance are in italics. Superscript numbers in first column indicate differences between patients in 1 CTP compared to those in the other 2. Differences between patients in CTP C versus CTP A and B:
↵1 p < 0.05,
↵3 p = 0.08. Differences between patients in CTP C versus CTP A and B:
↵2 p < 0.05. The last visit was the “12”-month visit for 43 patients, and “9”-month visit for 1 patient. For 2 patients, the chronological time of their “12”-month visit was at 274 or 295 days. We therefore decided to include a subject whose last study visit was the “9”-month visit, which occurred at a chronological time of 283 days. This patient lived far from the study site, did not make the “12”-month visit, but did have a later followup visit. Extracutaneous involvement was scored based on presence at any time during the study. Growth disturbance refers to limb girth or length difference, or facial or trunk hemiatrophy. Joint involvement refers to arthritis (swollen joint) or joint contracture. Other extracutaneous involvement were headache (4), seizure (1), pseudopapilledema (1), Raynaud phenomenon (2), muscle spasm (2), and dental crowding (1). The number of anatomic sites is based upon the divisions used for the LS Cutaneous Activity measure; the body is divided into 19 sites: scalp, face, neck, chest, abdomen, back, buttock, arm (right/left), forearm (right/left), hand (right/left), thigh (right/left), lower leg (right/left), and foot (right/left). The p values on the “Race” and “Subtype” rows refer to the significant difference found in the distribution of all the listed races or subtypes, respectively. Circumscribed morphea subtype was also found to differ between patients in CTP C versus CTP A and B (p = 0.004). Family history of an autoimmune disease includes rheumatic diseases and additional autoimmune diseases such as celiac disease, type 1 diabetes mellitus, and multiple sclerosis. Thirty-eight patients were evaluated for ANA positivity; 40 patients had an initial ESR or CRP test done. ANA: antinuclear antibody; ESR: sedimentation rate; CRP: C-reactive protein; CTP: consensus treatment plan; IQR: interquartile range; LS: localized scleroderma; NS: nonsignificant; PO: oral; MTX: methotrexate; IV: intravenous; CS: corticosteroids.