Session Information
Date: Monday, November 6, 2017
Session Type: ACR Poster Session B
Session Time: 9:00AM-11:00AM
Background/Purpose:
Juvenile localized scleroderma (jLS) is often associated with deep tissue and extracutaneous involvement (ECI), putting children at risk for severe morbidity such as hemiatrophy, arthritis, and seizures. Few studies have prospectively evaluated occurrence of ECI or its impact on jLS health related quality of life (HRQoL). Our multicenter, multidisciplinary group (LOCUS, Localized scleroderma Clinical and Ultrasound Study group) conducted a study to identify factors associated with ECI in jLS.
Methods:
We conducted a prospective observational cohort study of active and inactive jLS patients. Inclusion criteria included diagnosis of jLS by a pediatric rheumatologist or dermatologist, onset < 16 years, and willingness to complete questionnaires. Ethical board approval was obtained at each site.
At each of the 3 study visits, physicians scored clinical disease features and Physician Global Assessments of activity and damage. Parents scored two global assessments (100 mm visual analog scales): 1) Impact of disease on their child’s life at time of visit (Impact VAS, anchors No Impact, Very Large Impact), and 2) Disease activity (Activity VAS, anchors Not Active, Very Active). Parents also noted if specific symptoms or problems had occurred since the prior visit or in past 2 months. Physicians completed LS treatment and ECI forms. The ECI form was a checklist of specific problems for 8 organ systems, with option to list additional items.
Descriptive statistics, Spearman correlations, and least squares means were performed. Models were built by choosing variables that had a significant correlation with Impact VAS scores, followed by elimination of variables based on log-likelihood scores.
Results: Of the 90 subjects, 55.6% had ECI – mostly musculoskeletal including joint contracture (27), bone size difference (23), and myalgia (12). Factors associated with ECI included higher PGA-Damage and Impact VAS scores, mixed morphea subtype, limb involvement, RF positivity, and systemic treatment (Table). No association was found with gender, age of onset, disease duration, or ANA positivity. High impact VAS was associated with altered sensation at skin lesion site, joint problems, difficulty playing, and missing school (r= 0.209-0.322). Modeling to identify variables associated with higher Impact VAS scores identified female sex (OR 10.7, 95% CI 3.9 to 29.5, p <0.001), active disease (OR 2.9, 95% CI 1.2 to 6.8, p = 0.014), and ECI (OR 2.8, 95% CI 1.3 to 5.9, p = 0.008).
Conclusion: Most jLS subjects had ECI, which occurred within a few years of disease onset. Several associated factors were identified, and subjects with EC were found to have higher physician rated damage and parent rated impact scores. The odds of higher impact scores were strongly associated with female sex. There is a need for more study to better evaluate variables associated with development of ECI and its impact on HRQoL.
Characteristics of Subjects with and without ECI
|
All (90) |
+ ECI (50) |
No ECI (40) |
P value (+ vs No ECI) |
|
|
Gender: Female : Male |
70 : 20 |
39 : 11 |
31 : 9 |
NS |
|
Age at Disease Onset, median years, (IQR) |
7.9 (5.0, 10.0) |
7.7 (4.8, 9.8) |
8 (5.3, 10) |
NS |
|
Disease Duration at Study Entry, median years (IQR) |
2.75 (1.4, 5.0) |
3 (1.6, 5.3) |
2.2 (1.1, 5) |
NS |
|
Race: Caucasian African American Asian Mixed |
70 2 7 10 |
37 2 3 8 |
33 0 4 2 |
NS |
|
Ethnicity: Hispanic |
13 |
7 |
6 |
NS |
|
Active Disease Status |
66 |
37 |
29 |
NS |
|
LS Subtype: Circ superficial Circumscribed deep Linear trunk or limb Linear head Generalized morphea Mixed morphea |
4 12 31 10 7 26 |
0 5 18 3 4 20 |
4 7 13 7 3 6 |
0.005 |
|
Mixed Morphea Subtype |
26 |
20 |
6 |
0.007 |
|
Anatomic site: Head Neck or Trunk Limb |
15 28 48 |
6 14 32 |
9 14 16 |
0.046 |
|
Family History of Autoimmune disease |
53 |
35 |
18 |
0.021 |
|
RF positivity (denominator = number of subjects who were tested) |
6/33 |
6/14 |
0/19 |
0.011 |
|
Elevated IgG or IgE level (denominator = number of subjects who were tested) |
9/38 |
9/22 |
0/16 |
0.003 |
|
PGA-Activity (0-100 mm) |
21.1 |
21.4 |
18.2 |
NS |
|
PGA-Damage (0-100 mm) |
25.5 |
28.0 |
19.2 |
0.001 |
|
Impact VAS (0-100 mm) |
36.1 |
44.1 |
19.6 |
0.017 |
|
Activity VAS (0-100 mm) |
47.0 |
52.5 |
34.8 |
NS |
|
Corticosteroid Treatment Prior to Study Entry |
55 |
41 |
14 |
<0.001 |
|
Methotrexate Treatment Prior to Study Entry |
65 |
42 |
23 |
0.013 |
|
Corticosteroid Treatment During Study |
29 |
23 |
6 |
0.002 |
|
Methotrexate Treatment During Study |
51 |
34 |
17 |
0.031 |
To cite this abstract in AMA style:
Li SC, Andrews T, Chen M, Torok KS, Pope E, Stewart KG, Higgins GC, Rabinovich CE, Laxer RM, Haines K, Punaro M, Jacobe H, O'Neil K. Extracutaneous Involvement Is Common in Juvenile Localized Scleroderma and Associated with a Higher Level of Perceived Disease Impact [abstract]. Arthritis Rheumatol. 2017; 69 (suppl 10). https://acrabstracts.org/abstract/extracutaneous-involvement-is-common-in-juvenile-localized-scleroderma-and-associated-with-a-higher-level-of-perceived-disease-impact/. Accessed .« Back to 2017 ACR/ARHP Annual Meeting
ACR Meeting Abstracts - https://acrabstracts.org/abstract/extracutaneous-involvement-is-common-in-juvenile-localized-scleroderma-and-associated-with-a-higher-level-of-perceived-disease-impact/