Session Information
Session Type: ACR Poster Session B
Session Time: 9:00AM-11:00AM
Background/Purpose: Children with juvenile myositis (JM) have a variable disease course, in part associated with myositis specific/associated antibodies (MSA). Time to off medications has not been well documented. Published reports show 18-55% develop calcifications, but little is known about disease course and damage after 60 observation months. Objective: To examine the records of children with JM at specific time points over 5 years to determine disease activity, therapy duration and evidence of damage.
Methods: After obtaining informed consent, 102 children were enrolled. 65 were treated elsewhere then transferred care (TR). 37 were diagnosed at our center, untreated at first visit (UTR). MSAs (Oklahoma Medical Research Foundation Clinical Immunology Laboratory) were determined by immunodiffusion and immunoprecipitation. Disease Activity Scores (DAS) for skin and muscle involvement, nailfold capillary end row loop (ERL) number, reported cataracts and BMI were obtained at months 0 (first visit to our center) 6, 12, 24, 36 and 60, as well as periodic bone density (DXA) evaluation. Data was analyzed using ANOVA or t tests.
Results: Table 1 describes patient features. TR and UTR groups were similar for race and age at disease onset. TR patients presented after a mean of 12.7 months of treatment (SD 17.23). At first visit, the DAS was essentially equivalent for both groups but at 60 months was significantly lower for UTR (p=0.04). Mean time to off medication was 3.8 years (SD 1.54). Calcifications were present in 4 (10.8%) UTR and 8 (12.3%) TR. Cataracts were reported in 13 (35.1%) UTR and 19 (29.2%) TR. TR had a lower average lumbar Z-score at months 0 and 60 than UTR, though not statistically significant. At 60 months, the mean BMI percentile of each group was 66th. ERL number improved in both groups, approaching the normal value of 7. MSA distribution was: p155/140 (29.4%), none (27.5%), Mi-2 (5.9%), MJ (3.9%), MDA5 (1%), Ro (2.9%), Ro plus another MSA (6.9%), three antibodies (12.7%) and antibodies seen in overlapping connective tissue disease ± MSA (9.8%). TR patients with ≥1 MSA trended to have the lowest lumbar Z-score (p=0.085) but groups were too small for further analysis.
Conclusion: Between TR and UTR patients, there were few differences at first visit and at 60 months. TR patients had a higher DAS total and muscle at 60 months, possibly reflecting more severe disease, thus prompting referral to our center. MSA distribution was similar to previously reported frequencies but only 11.8% had calcifications, much less than previously reported. Mean lumbar spine Z-scores were below average for age and cataracts occurred in 31.4% of patients, supporting the need for steroid-sparing JM treatments. Our study describes a large JM patient cohort through 60+ treatment months, a middle-term outcome rarely reported in JM literature. Table 1. Disease Features of Untreated and Treated JM patients at first visit to the Cure JM Myositis Clinic (month 0) and 60 months
|
Untreated patients, mean (SD) [n=x] total N=37 |
Treated patients, mean (SD) [n=x] total N=65 |
p value |
All patients, mean (SD) [n=x] total N=102 |
|
|
Age of disease onset (years) |
5.64 (2.87) [n=37] |
6.59 (4.15) [n=65] |
NS |
6.24 (3.75) [n=102] |
|
DAS total month 0 (maximum 20) |
10.31 (3.30) [n=35] |
10.66 (4.1) [n=64] |
NS |
10.54 (3.81) [n=99] |
|
DAS total month 60 (maximum 20) |
1.96 (2.53) [n=37] |
3.14 (3.14) [n=63] |
0.04 |
2.71 (2.98) [n=100] |
|
DAS muscle month 60 (maximum 11) |
0.45 (1.08) [n=37] |
0.95 (1.78) [n=63] |
NS |
0.77 (1.57) [n=100] |
|
DAS skin month 60 (maximum 9) |
1.51 (2.21) [n=37] |
2.19 (2.32) [n=63] |
NS |
1.94 (2.29) [n=100] |
|
Time to medication discontinuation (years) |
4.12 (1.88) [n=28] |
3.53 (0.98) [n=26] |
NS |
3.84 (1.54) [n=54] |
|
Lumbar Z-score at month 0 |
-0.18 (1.16) [n=17] |
-0.48 (1.19) [n=42] |
0.027 |
-0.39 (1.18) [n=59] |
|
Lumbar Z-score at month 60 |
-0.19 (1.01) [n=28] |
-0.57 (1.00) [n=46] |
0.013 |
-0.42 (1.01) [n=74] |
|
BMI percentile at month 0 |
53.64 (23.87) [n=14] |
70.82 (28.58) [n=29] |
NS |
65.23 (28.06) [n=43] |
|
BMI percentile at month 60 |
66.6 (31.29) [n=30] |
66.38 (29.11) [n=59] |
NS |
66.46 (29.68) [n=89] |
|
ERL number at month 0 |
4.45 (1.12) [n=36] |
4.48 (1.21) [n=62] |
NS |
4.47 (1.17) [n=98] |
|
ERL number at month 60 |
5.93 (1.15) [n=33] |
5.63 (1.29) [n=62] |
NS |
5.73 (1.25) [n=95] |
To cite this abstract in AMA style:
Pachman LM, Curran ML, Morgan GA, Amoruso MC, N. Targoff I, Huang CC. A Five Year Study of 102 Children with Juvenile Myositis: Disease Course and Outcomes [abstract]. Arthritis Rheumatol. 2016; 68 (suppl 10). https://acrabstracts.org/abstract/a-five-year-study-of-102-children-with-juvenile-myositis-disease-course-and-outcomes/. Accessed .« Back to 2016 ACR/ARHP Annual Meeting
ACR Meeting Abstracts - https://acrabstracts.org/abstract/a-five-year-study-of-102-children-with-juvenile-myositis-disease-course-and-outcomes/