Table 4

Summary of items included in the JDM optimal dataset, form B (completed at every visit representing status of the patient at the current time point)

Section headingItemsAdditional items conditional on previous response (summary)
Growth1Height of patient (in centimetres)
2Weight of patient (in kilograms)
Muscular involvement3Presence of symmetrical proximal muscle weakness
4Childhood Myositis Assessment Scale scoreState score (out of 52)
5Manual Muscle Testing scoreState score (out of 80)
6VAS score for global muscle disease activityIf measured, mark score on 10 cm line*
Skeletal involvement7Arthritis due to myositis
8Joint contractures due to myositis
9VAS score for global skeletal disease activityIf measured, mark score on 10 cm line*
Cutaneous involvement10Gottron’s papules or Gottron’s sign
11Heliotrope rash
12Periungual capillary loop changes (plus measure of capillary density if available)
13Malar or facial erythema
14Linear extensor erythema
15‘V’ sign
16Shawl sign
17Non sun-exposed erythema
18Extensive cutaneous erythema, which may include erythroderma
19Livedo reticularis
20Cutaneous ulceration
21Mucus membrane lesions
22Mechanic’s hands
23Cuticular overgrowth
24Subcutaneous oedema
25Panniculitis
26Alopecia (non-scarring)
27Calcinosis (with active disease)
28VAS score for global cutaneous disease activityIf measured, mark score on 10 cm line*
Features suggestive of myositis overlap29Does this patient have a myositis overlap condition?If yes, asked to select all that apply (four options)
30Raynaud’s phenomenon
31Sclerodactyly
Gastrointestinal involvement32Dysphagia due to myositis
33Abdominal pain due to myositis
34Gastrointestinal ulceration due to myositis
35VAS score for global gastrointestinal disease activityIf measured, mark score on 10 cm line*
Pulmonary involvement36Pulmonary involvement/respiratory muscle weakness or interstitial lung disease due to myositis
37Dysphonia due to myositis
38VAS score for global pulmonary disease activityIf measured, mark score on 10 cm line*
Cardiovascular involvement39Cardiovascular involvement due to myositis
40BP recordingState systolic and diastolic measurement
41BP elevated suggesting hypertension (for age of patient)
42VAS score for global cardiovascular disease activityIf measured, mark score on 10 cm line*
Constitutional features43Fever (>38°C) due to myositis
44Weight loss (>5%) due to myositis
45Fatigue due to myositis
46VAS score for global constitutional disease activityIf measured, mark score on 10 cm line*
Global disease assessment by clinician47Physician VAS score of global disease activityIf measured, mark score on 10 cm line*
48Physician VAS score of extramuscular disease activityIf measured, mark score on 10 cm line*
Global disease assessment by patient/parent49Patient/parent VAS score for global disease activityIf measured, mark score on 10 cm line* and state who completed (four options)
50Patient/parent VAS score for painIf measured, mark score on 10 cm line*
PROM51Use of an age-appropriate PROM of functionAsked to state PROM used and score
52Use of an age-appropriate patient/parent-reported measure of quality of lifeAsked to state PROM used and score
Investigations53Elevation of any muscle enzyme (including CPK, LDH, aldolase, AST/SGOT, ALT/SGPT) above normal rangeIf elevated, asked to select which apply (five options)
Specimens available54Has this patient had specimens taken that may be available for specific research projects? This may include DNA, serum, biomarkers, biopsy tissue or other materialIf answer is ‘yes’, asked to select which apply (three options)
Treatment55Is the patient on treatment (now or since last visit)?Asked to select all that apply (16 options) and to state dose, route and frequency for each medication
56Is the patient doing a regular exercise routine prescribed by a healthcare professional aimed at improving/maintaining:
(A) range of movement?
(B) muscle strength?
  • *0 is inactive or lowest score and 10 is most active or highest score on 10 cm VAS scores.

  • ALT, alanine transaminase; AST, aspartate aminotransferase; BP, blood pressure; CPK, creatine phosphokinase; JDM, juvenile dermatomyositis; LDH, lactate dehydrogenase; PROM, patient/parent-reported outcome measure; SGOT, serum glutamic oxaloacetic Transaminase; SGPT, serum glutamic-pyruvic transaminase; VAS, Visual Analogue Scale.