Discordance Between Patient or Family and Physician Assessments Common in Juvenile Dermatomyositis

doctors in waiting room
Researchers measured discordance between patient or family and physician global activity scores in juvenile dermatomyositis, and determined factors associated with discordance.

Discordance in global disease activity scores between patient or family and physician assessments may be common in juvenile dermatomyositis, according to study results published in Pediatric Rheumatology. Researchers indicated that a higher patient or family rating was linked to poorer scores of patient-reported outcomes, and a higher physician rating was linked to poorer objective measures.

To measure discordance between patient or family and physician assessments of the disease, researchers collected data, including demographics, medication history, patient-reported outcome measures reported by patients or family members, and physician-reported outcome measures from baseline visit forms. Measures of patient-reported outcomes were global disease activity assessment on a 10-point visual analog scale (VAS), the Childhood Health Assessment Questionnaire (CHAQ), overall pain score using the VAS, or Faces of Pain Scale using the Likert scale; physician-reported outcomes were measured by global activity assessment on the VAS, measurement of proximal muscle weakness using the Likert scale, muscle enzyme testing, findings from examinations, and associated comorbidities.

The primary outcome of the study was patient/parent and physician global activity assessment scores based on the 10-point VAS.

Of 639 patients enrolled in the Childhood Arthritis and Rheumatology Research Alliance Legacy Registry from 2010 to 2015, researchers evaluated data for 563 patients with physician-diagnosed juvenile dermatomyositis (mean age, 10.6 years; age at disease onset, 5.5 years), with available patient or family and physician global VAS scores.

Results of the study indicated that 61% (n=344) of patient or family and physician global VAS scores were concordant, 26% (n=149) were discordant with patient or family rating of VAS scores ≥2 points greater than that of physician rating, and 12% (n=70) were discordant with physician rating of VAS scores worse than that of patient or family rating. Of the discordant scores, 68% of patients or family members rated disease activity as worse compared with physician ratings, and 32% of physicians rated higher disease activity compared with patient or family rating.

Researchers reported significantly worse CHAQ scores and more frequently reported poor quality of life (P <.01) in patients when patient or family VAS scores were ≥2 points above that of physician VAS scores; however, data indicated more muscle enzyme abnormalities, worse muscle weakness, rash, nailfold changes, calcinosis, higher percentage of joint involvement, and steroid treatment (all P <.01) in patients.

Medication use and demographic factors were not associated with patient or family members and physician assessments.

In cases in which patient or family VAS scores were ≥2 points greater than physician scores, multivariate analysis of discordance in global activity assessment showed independent predictors of calcinosis and lower quality of life (good/very good vs excellent: odds ratio [OR], 12.5 [95% CI, 4.5-25.0; P <.001]; and poor vs excellent: OR, 17.0 [95% CI, 4.6-32.0; P <.001]). Cases in which physician VAS scores were ≥2 points greater than patient or family scores showed significant independent predictors, including rash (OR, 11.0; 95% CI, 3.7-22; P <.001), calcinosis (OR, 3.3; 95% CI, 1.4-8.2; P =.009), nailfold changes (OR, 2.1; 95% CI, 1.1-4.3, P =.040), and steroid treatment (OR, 2.3; 95% CI, 1.3-5.0, P =.039).

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Study limitations included difficulty in assessing the effectiveness of treatment and the concern that significant discordance in global activity assessment could be associated with a lower level of satisfaction and decreased treatment regimen adherence among patients or family members.

“Our work underscores the need to develop alternative relevant and valid patient-focused outcome measures that can be integrated into our overall assessment of patients with [juvenile dermatomyositis], for use not only in clinical trials, but also in clinical decision-making and routine care of patients with [juvenile dermatomyositis] to improve future outcomes from this disease,” the researchers concluded.


Tory H, Zurakowski D, Kim S; and the CARRA Juvenile Dermatomyositis Quality Measures Workgroup for the CARRA Registry Investigators. Patient and physician discordance of global disease assessment in juvenile dermatomyositis: findings from the Childhood Arthritis & Rheumatology Research Alliance Legacy Registry. Pediatr Rheumatol. 2020;18:5.