Table 3B.

Change in cutaneous, physician, and parent/patient scores from baseline to last visit in each CTP: censored for treatment failures (n = 33).

Measure	Visit	All Patients, n = 33	A: MTX, n = 8	B: MTX + IV CS, n = 17	C: MTX + PO CS, n = 8
PGA-A	Baseline	4 (3–6)	3.5 (2–6)	5 (4–6)	4 (3.5–5.5)
PGA-A	Last	0 (0–1)^*	0.5 (0–1.5)^*	0 (0–1)^*	0 (0–2)^*
mLoSSI	Baseline	8 (4–10)	9 (3–14.5)	9 (5–11)	5 (4.5–6)
mLoSSI	Last	0 (0–1)^*	0 (0–1.5)^*	0 (0–1)^*	0 (0–0)^*
LSCAM	Baseline¹	7 (4–12)	10.5 (6.5–14)	8 (4–14)	4 (3.5–5)
LSCAM	Last¹	1 (0–2)^*	1.5 (0–3)^*	1 (1–3)^*	0 (0–1)^*
PGA-D	Baseline	4 (3–5)	3.5 (2.5–4.5)	4 (3–6)	3 (2.5–5.5)
PGA-D	Last	3 (2–4)	2 (1–3.5)	4 (2–5)	2.5 (1–3)
LoSDI	Baseline	7 (3–13)	10.5 (5.5–17)	6 (3–13)	5.5 (2.5–7.5)
LoSDI	Last	7 (4–11)	7 (4–17.5)	9 (5–11)	4.5 (2.5–9)
LSDam	Baseline	9 (5–16)	16 (7–21.5)	8 (5–16)	7 (3.5–9.5)
LSDam	Last	8 (5–11)	8 (4.5–19)	10 (5–15)	5.5 (2.5–9)
Overall global health	Baseline	2 (1–2)	1.5 (1–2)	2 (1–2)	2 (1–2)
Overall global health	Last	2 (2–2)	2 (1–2)	2 (2–2)	2 (2–2)
Impact of rheumatic disease	Baseline	1 (0–2)	0 (0–1)	1 (0–5)	1 (0–3)
Impact of rheumatic disease	Last	1 (0–2)	1 (0–1)	2 (1–2)	0.5 (0–1.5)

Values shown are median (IQR). Significant changes in scores from baseline to last visit are indicated in All Patients and CTP columns:
↵^ p < 0.01.
↵* p < 0.001.
Superscript numbers in Visit column indicate differences in scores between patients in CTP C versus those in CTP A and B:
↵1 p < 0.05. Patients who dropped out or discontinued treatment prior to their 9-month visit (n = 6) were not included. Patients who deviated from their initial CTP because of adverse event but were able to continue systemic treatment were included in Table 3A. The mLoSSI and LSCAM score cutaneous activity features, while the LoSDI and LSDam score cutaneous damage features. Overall global health refers to the parent or patient question, “How do you rate your child’s health?” (range: excellent = 5, to very poor = 1). If the child was answering the question, then the question was reworded to focus on the patient’s point of view. Effect of rheumatic disease refers to the parent or patient question, “Considering all the ways that your child’s rheumatic condition affects your child, rate how your child is doing” (0 = no impact, 10 = highest impact). Additional health-related quality of life measures asked of parents or patients included global pain (“How much pain do you think your child had because of his/her rheumatic condition in the past week?”) and the Childhood Health Assessment Questionnaire³³, neither of which were found to differ significantly between the baseline and last visit. Other collected measures were PedsQL general, PedsQL rheumatology, PedsQL Family Impact, and Children’s Dermatology Life Quality Index^34,35,36,37. CS: corticosteroid; CTP: consensus treatment plan; IQR: interquartile range; IV: intravenous; LoSDI: Localized Scleroderma Damage Index; LSCAM: Localized Scleroderma Cutaneous Activity Measure; LSDam: Localized Scleroderma cutaneous Damage measure; mLoSSI: modified Localized Scleroderma Severity Index; MTX: methotrexate; PedsQL: Pediatric Quality of Life Inventory; PGA-A: physician’s global assessment of disease activity; PGA-D: PGA of disease damage; PO: oral.