Session Type: ACR Poster Session B
Session Time: 9:00AM-11:00AM
Background/Purpose: All previous studies of disease outcome in juvenile dermatomyositis (JDM) patients have been based on retrospective data or cross sectional examination. We aimed to examine if disease activity, muscle strength and organ damage changed over time in an unselected cohort of JDM patients reassessed after long-term follow-up.
Methods: Patients who were included in a cross-sectional examination in 2005-2008 (visit 1) were invited for a re examination in 2013-2015 (visit 2). Patients were divided in active and inactive disease by the Paediatric Rheumatology International Trials Organisation (PRINTO) criteria. Disease Activity Score (DAS) was used to measure disease activity in skin and muscle, while the myositis damage index (MDI) was used to assess cumulative organ damage. The manual muscle test (MMT) and child myositis assessment scale (CMAS) were used to measure muscle strength/endurance, respectively. Also, use of anti-inflammatory medication was assessed.
Results: 42 patients (62% female) participated in both visits. Duration between the visits was mean 7.5 (1.0) years, and disease duration from symptom onset was median 21.5 years, (range 7.6-42.7). No difference in cumulative organ damage, muscle strength / endurance or disease activity measures was found between the visits, except that DAS muscle was higher in visit 2 (Table 1). By the PRINTO criteria, 25(60%) had inactive disease at visit 2; 4 of those used anti-inflammatory medication. Of the 17 (40%) active patients, only 3 used anti-inflammatory medication. 29 patients remained in the same PRINTO category at both visits (18 inactive and 11 active), whereas 7 active patients became inactive and 5 inactive patients became active between the visits.
Conclusion: Despite 40 % of JDM patients still had active disease after median 21.5 years follow-up and the majority of active patients were not on anti-inflammatory medication, no increase in cumulative organ damage was found over a 7.5 years time period. Table 1: Disease characteristics in 42 JDM patients at visit 1 and visit 2
|Visit 1||Visit 2||P value|
|Follow-up time, years||13.4 (2.0-34.6)||21.5 (7.6-42.7)||NA|
|DAS muscle (0-11)||1.2 (1.2)||1.8 (1.6)||0.007|
|DAS skin (0-9)||3.3 (2.2)||2.7 (2.0)||NS|
|DAS total (0-20)||4.5 (2.7)||4.5 (2.6)||NS|
|MDI (0-40)||3.5 (2.5)||3.2 (1.9)||NS|
|PRINTO inactive, n (%)||23 (56)||25 (60)||NS|
|CMAS (0-52)||50 (28-42)||49 (39-52)||NS|
|MMT-8 (0-80)||79 (61-80)||78 (50-80)||NS|
|On prednisolone, n (%)||6 (14)||3 (7)||NS|
|On methotrexate, n (%)||9 (21)||7 (17)||NS|
Numbers are mean (SD) or median (range) if not otherwise stated; NA, not assessed; NS, non-significant
To cite this abstract in AMA style:Berntsen KS, Flatø B, Sjaastad I, Sanner H. In Juvenile Dermatomyositis, Organ Damage Is Comparable after Median 13.5 and 21.5 Years Follow-up Time, Despite Sustained Disease Activity [abstract]. Arthritis Rheumatol. 2016; 68 (suppl 10). https://acrabstracts.org/abstract/in-juvenile-dermatomyositis-organ-damage-is-comparable-after-median-13-5-and-21-5-years-follow-up-time-despite-sustained-disease-activity/. Accessed May 20, 2019.
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