Table 2 Summary of questions asked in clinician survey compared to the parent and patient questionnaires

General format of questionnaire

Two separate sections: Section A – when patient seen for their first visit only; Section B – all visits (first and subsequent). Asked to rate importance of each variable on a 1–9 scale separately for clinical use and for research

Asked to rate: How important do you think each one of these is when thinking about how well or unwell your child is due to JDM? (“not that important”/ “important”/or “really important”). Not asked separately about clinical/research

Asked to rate: How important do you think each one of these is when thinking about how well or unwell you are due to JDM (“not that important”/“important”/“really important”) Not asked separately about clinical/research

General questions on collecting and storing information and/or specimens

N/A

Asked to rate how important they think it is for all doctors and nurses/therapists to collect key information in the same way when they are looking after children with juvenile dermatomyositis (JDM)

Asked to rate how important they think it is for all doctors and nurses/therapists to collect key information in the same way when they are looking after children with JDM

How important do you rate storing specimens at diagnosis for other biomarkers? (eg., DNA/serum/any other material)^a

Asked to rate how important they think it is to store the type of information collected in clinic in a research database

Asked to rate how important they think it is to store the type of information collected in clinic in a research database

Demographics of patient

Date of birth

N/A

N/A

Gender

Race

Ethnicity

Family history

Of autoimmune disease

N/A

N/A

Of neuromuscular disease

Diagnostic data (general)

Age of onset of JDM

N/A

N/A

Age of diagnosis of JDM

MUSCLE – Diagnostic/activity data

Presence of symmetrical proximal muscle weakness: at diagnosis (section A) and every visit (section B)

Asking you if your child has any weakness

Asking if you have any weakness

Presence of other muscle weakness (eg., distal or asymmetrical): section B only

Use of a validated tool to score muscle strength and/or function (options for different muscle strength scores)^a

Testing to see how strong your child is

Testing to see how strong you are

SKIN – Diagnostic/activity data

Heliotrope rash (sections A and B)

Asking you if your child has any skin rashes

Asking if you have any skin rashes

Gottron’s papules/Gottron’s sign (sections A and B)

Nail-fold capillary changes (sections A and B)

Other characteristic JDM rash

Use of a validated skin tool for JDM (options given to choose specific tools)^a

Looking for rashes or skin signs that may suggest active JDM

Looking for rashes or skin signs that may suggest active JDM

SKIN – Additional activity data asked in section B only

Lipodystrophy

N/A

N/A

Calcinosis

Cutaneous ulceration

Subcutaneous oedema

Malar/facial erythema

Shawl sign/V-sign

Mechanic’s hands

Alopecia

Vasculopathic lesions

Photosensitivity

Livido reticularis

Other erythema

Panniculitis

MAJOR ORGAN INVOLVEMENT due to myositis – asked in section B only of clinician survey, plus patient/parent questionnaires

Musculoskeletal involvement (specific questions added for arthritis/contractures)^a

Asking if your child has any muscle or joint pains

Asking if you have any muscle or joint pains

Looking for swelling in any of the joints

Looking for swelling in any of the joints

Gastrointestinal involvement (specific questions added for dysphagia/abdominal pain or GI ulceration)^a

Asking if your child has any difficulty with swallowing/eating or if they have tummy pain

Asking if you have any difficulty with eating or if you have tummy pain

Pulmonary involvement suggesting interstitial lung disease

Asking if your child has any shortness of breath or chest pain that may be related to JDM

Asking if you have any shortness of breath or chest pain that may be related to JDM

Cardiac involvement

CONSTITUTIONAL SYMPTOMS due to myositis – asked in section B only of clinician survey and in patient/parent questionnaire

Fever (>38 °C) due to myositis

N/A

N/A

Weight loss (>5 %) due to myositis

N/A

N/A

Fatigue due to myositis

Asking if your child feels tired due to JDM

Asking if you feel tired due to JDM

Asking how tired your child feels using a formal “fatigue scale”

Asking how tired you feel using a formal ‘fatigue scale’

Irritability due to myositis

Asking if your child feels irritable or miserable due to JDM

Asking if you feel irritable or miserable due to JDM

Raynaud’s phenomenon

Asking if your child gets colour changes in their hands in cold weather

Asking if you get colour changes in your hands in cold weather

Growth

Height of patient (section B only)

Checking how well your child is growing (height)

Checking how well you are growing

Weight of patient (section B only)

Asking about any weight loss and checking your child’s weight

Asking about any weight loss and checking your weight

Development/puberty

N/A

Asking about how your child is developing or how puberty is progressing

Asking about how you are developing or how puberty is progressing

Other information

Presence of malignancy (section B only)

N/A

N/A

Patient having ongoing follow-up at this centre? (If no: options given for reason why)^a

Global disease activity

Use of a physician-scored measure of global disease activity - such as physician Visual Analogue Scale; VAS (specific choices for activity scores given)^a

Asking your doctor to mark on a 0–10 cm scale how well or unwell they think your child has been based on what you tell them and what they see at your clinic visit

Asking your doctor to mark on a 0–10 cm scale how well or unwell they think you have been

Patient/parent Reported Outcome Measures (PROMs), Quality of Life/School issues

Use of a patient/parent reported outcome measure of disease activity or use of a validated measure of function (such as patient VAS/CHAQ) and/or a tool to measure Quality of Life (QoL) (specific choices given)^a

Asking you or your child to complete a questionnaire that looks at how easy or difficult it is for them to do things like get dressed, have a bath, do activities

Asking you or to complete a list of questions that look at how easy or difficult it is for you to do things like get dressed, have a bath, do activities

Asking you/your child to mark on a 0–10 cm scale how well or unwell your child has been over the last 4 weeks

Asking you to mark on a 0–10 cm scale how well or unwell you have been over the last 4 weeks due to JDM

Asking you/your child to mark on a 0–-10 cm scale how much pain your child has had in the last 4 weeks due to JDM

Asking you to mark on a 0–10 cm scale how much pain you have had in the last 4 weeks due to JDM

Asking how many days your child has missed school or college due to JDM

Asking how many days you have missed school or college due to JDM

Asking more questions about your child’s school – how are things? Are they able to keep up with peers?

Asking more questions about school – how are things? Are you able to keep up with your peers?

Asking your child how they feel emotionally in relation to their JDM (questions relating to Quality of Life or mood)

Asking about your feelings in relation to your JDM

INVESTIGATIONS: diagnostic data/disease activity data

Elevation of muscle enzymes at diagnosis (section A) or later in disease course (section B). (Questions asked about which specific enzymes to measure)^a

Taking blood tests to monitor how active the disease is

Taking blood tests to monitor how active the disease is

Electromyography (EMG) changes of myositis at diagnosis (section A)

Muscle biopsy evidence of myositis at diagnosis (section A)

Magnetic Resonance Imaging (MRI) changes of myositis at diagnosis (section A)

Asking your child to have a scan such as an MRI scan of their muscles to monitor the disease

Asking you to have a scan such as an MRI scan of your muscles

Section B only: abnormal investigations (imaging, histology or cardio-pulmonary function tests indicating flare of myositis). If “yes,” tick which investigations are abnormal

Anti-nuclear Antibody (ANA) positivity at diagnosis (section A only)

Myositis specific antibody (MSA) positive at diagnosis (section A only)

Myositis associated antibody (MAA) positivity at diagnosis (section A only)

Treatment

Date treatment started (mm/yyyy)

Asking specific questions about your child’s medicines and how they make them feel? Any unwanted effects?

Asking specific questions about your medicines and how they make you feel? Any unwanted effects?

Patient taking steroids? Yes/No (options for type of steroid given)^a

Asking you about the medicines your child is taking at the moment

Asking you about the medicines you are taking at the moment

Patient taking a Disease Modifying Anti-Rheumatic Drug (DMARD)? (Not including biologic DMARDS) Yes/No (with option to select name of drug from a list)

Patient taking a biologic? Yes/No (with option to select name of drug from a list)

Patient having physiotherapy and/or occupational therapy?

Asking you if your child is doing any physiotherapy exercises

Asking you if you are doing your physiotherapy exercises

Other questions

To determine practice and experience - primary role, patient group, number of paediatric/adult patients under their care, geographical region of practice and membership of societies/professional bodies

How would you prefer information to be asked of you? (eg., in clinic, questionnaire before clinic, questionnaire before you come to clinic)

How would you prefer information to be asked of you? (eg., in clinic, questionnaire before clinic, questionnaire before you come to clinic)

Further information

Are there any variables that you think are important for clinical care that are not currently included in the list above? If so, please state what these are (same question also asked separately for variables important for research)

Are there any other key questions that we have missed but that you think it is important for us to ask when you come to clinic, thinking about how well/unwell you/your child is? If so, what key questions are they?

Are there any other key questions that we have missed but that you think are important for us to ask when you come to clinic, thinking about how well or unwell you are? If so, what key questions are they?

Is there anything in this list (or from your experience of bringing your child to clinic) that you do not think should be included or you think your child does not like? If so, what? Why do you think that these questions should not be included?

Is there anything in this list (or from your experience of coming to clinic) that you do not like? If so, what? Why do you think that these questions should not be included?