Session Information
Session Title: Pediatric Rheumatology - Clinical and Therapeutic Aspects: Pediatric Lupus, Scleroderma and Myositis (ACR)
Session Type: Abstract Submissions (ACR)
Background/Purpose : Three types of disease courses can be distinguished in patients with juvenile idiopathic inflammatory myopathies (JIIM), namely monocyclic (M), polycyclic (P), and chronic (C). This study explored the association of demographics, clinical onset features and myositis autoantibodies with disease course in a large JIIM cohort.
Methods : In the present study, we included 365 patients with JIIM (295 dermato-, 28 poly-, and 42 overlap myositis) which were enrolled into IRB-approved studies in the US and Canada and had a disease duration > 2 years from diagnosis. Blood samples and physician questionnaires with demographics and clinical onset features were obtained. Myositis autoantibodies were determined by immunoprecipitation and blotting methods. Follow-up was performed through medical record review. The disease course classification was: M – no active disease and off medication within 2 years of diagnosis (n=88); P – disease recurrence after definite remission (n=86); and C – persistent disease or continuation of medication for > 2 years (n=191). Parameters with p<0.10 in univariate analysis were analyzed by multinomial logistic regression.
Results : Factors significant only in univariate analysis were cuticular overgrowth (M < P and C), V- and/or shawl-sign rash (M and P < C), contractures (P < C and M), photosensitivity (M < C), dyspnea at rest (P < C), palpitations and/or syncope (M and C < P), geoclimactic zone, and anti-Ro autoantibodies (M < C). Abnormal aldolase was more frequent in P compared to M and C, but not included in multivariable analysis due to missing values. Two different multinomial logistic regression analyses with non-overlapping parameters were performed (see Table 1 for results). Myositis -specific and -associated autoantibodies were more frequent in P and C than in M, with the anti-p155/140 autoantibody as the most significant (p<0.0001). Documented infections within 6 months prior to illness onset were also more frequent in the P and C compared to M. P had less early illness signs and symptoms (=lower clinical symptom score) compared to M and C. A more severe illness onset was present in C compared to M and P. Compared to M, mucous membrane involvement was less frequent in C and distal weakness was less frequent in P and C. Weight loss was more frequent in C compared to P.
Conclusion : Myositis -specific and -associated autoantibodies (especially the anti-p155/140 autoantibodies) and selected features at illness onset were associated with a chronic course of illness in patients with JIIM. These findings suggest that certain predictors of poor prognoses can be identified that might influence treatment options at illness onset.
Table 1. Results of 2 separate multinomial logistic regression analyses.
|
Significant parameters |
p |
|
OR (95% CI) |
||||
|
|
|
|
P vs. M |
|
C vs. M |
|
C vs. P |
|
Significant parameters from analysis 1 |
|
|
|
|
|
|
|
|
Any myositis specific autoantibodies |
<0.0001 |
|
2.7 (1.3 to 5.7) # |
|
4.0 (2.1 to 7.6) # |
|
NS |
|
Any myositis associated autoantibodies |
<0.01 |
|
3.8 (1.2 to 11.9) † |
|
4.3 (1.6 to 12.0) # |
|
NS |
|
Any infection 6 months prior to illness onset |
<0.01 |
|
5.1 (2.0 to 12.7) # |
|
2.4 (1.0 to 5.8) † |
|
0.5 (0.2 to 1.0) † |
|
Clinical symptom score $ |
0.01 |
|
0.02 (0.0-0.8) † |
|
NS |
|
81 (4 to 1686) # |
|
Significant parameters from analysis 2 |
|
|
|
|
|
|
|
|
Anti-p155/140 autoantibodies |
<0.0001 |
|
3.5 (1.2 to 10.1) † |
|
7.1 (2.7 to 19.0) # |
|
2.1 (0.9-4.5) * |
|
Severity of illness at onset |
<0.01 |
|
NS |
|
3.3 (1.5 to 7.3) # |
|
2.2 (1.0 to 4.9) * |
|
Mucous membrane involvement |
0.02 |
|
NS |
|
0.3 (0.1 to 0.7) # |
|
NS |
|
Distal weakness |
0.04 |
|
0.4 (0.2 to 0.8) † |
|
0.5 (0.2 to 1.0) * |
|
NS |
|
Weight loss |
0.03 |
|
NS |
|
NS |
|
3.1 (1.3 to 7.6) † |
Data was adjusted for date of diagnosis and for the significant parameters in the analysis.
*p≤0.10
†p≤0.05
#p≤0.01
$ Odds ratio: per 0.01 increase in clinical symptom score
Disclosure:
G. E. A. Habers,
None;
A. M. Huber,
None;
G. Mamyrova,
Cure JM Foundation,
2;
I. Targoff,
None;
C. Boonacker,
None;
M. van Brussel,
None;
F. W. Miller,
None;
L. G. Rider,
None;
A. van Royen-Kerkhof,
None.
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ACR Meeting Abstracts - https://acrabstracts.org/abstract/anti-p155140-autoantibodies-and-selected-features-at-illness-onset-are-associated-with-a-chronic-course-of-illness-in-the-juvenile-idiopathic-inflammatory-myopathies/