Cure JM Family Survey: Early Diagnosis Initiative
Summary of Responses: Jan. – March 2016
Number of Respondents
403 respondents representing 18 countries and 47 U.S. states
Gender
- 73% of patients are female
- 27% of patients are male

Type of Myositis
- 98% have JDM versus JPM

Age of Diagnosis
- 37% were diagnosed between the ages of 3-5
- 28% were diagnosed between the ages of 6-8
- Only 10% were 13 and older and 10% were 2 and under
- 75% of patients are 8 and under

Muscle-Related Symptoms Prior to Diagnosis
- Top Response:
70% complained of being tired more often - More than 50% of patients also had:
- Difficulty climbing stairs
- Loss of ability to do physical abilities, i.e. bike riding, running, soccer
- Fatigue before normal and/or after physical activities
- Complaints of aches/pains
- Difficulty in keeping up with friends at school and in sports
- More than 40% of patients:
- Asked to be carried more often
- Difficulty walking
- Fell more frequently
- 26% had stomach discomfort and pain
Skin-Related Symptoms Prior to Diagnosis
- Top 2 Responses:
68% reported rash on cheeks and/or bridge of nose
56% reported red or purple coloring on eyelids - More than 50% of patients cited:
- Overall redness on hands (joints, knuckles, nail beds)
- Redness and/or bumps on knees
- Redness and/or bumps on elbows
- More than 40% reported:
- Redness and/or bumps on knuckles
- Red/swollen fingernails/cuticles
- Gottron’s papules
General Symptoms Prior to Diagnosis
- 84% reported fatigue
- 48%: sleeping more often
- 40%: low-grade fevers
- 35%: weight loss
- 25%: difficulty with bowel movements
- 25%: contractures
- 10%: lung issues
- 7%: lipodystrophy
- 6%: heart issues
Types of Doctors Consulted Prior to Diagnosis
- 81% consulted with their pediatrician
- 47% consulted with their dermatologist
Tests Used Prior To/During Diagnosis
- 97% bloodwork
- 65% MRI
- 63% strength testing
- 33% muscle biopsy
- 27% skin biopsy
Number of Times Patient Consulted a Doctor Prior to Referral to Specialist and/or Diagnosis
- 33% of patients saw 2-3 doctors prior to diagnosis or referral
- 29% of patients saw 6 or more doctors prior to diagnosis or referral

Type of Doctor who Ultimately Made the Diagnosis
- Rheumatologists made the diagnosis in 60% of the cases.
- Dermatologists made the diagnosis in 16% of the cases.
- Pediatricians made the diagnosis in 7% of the cases.
Did Patient Have Infection or Virus Within 3 months of Onset of Symptoms?
- 49% of patients had an infection or a virus within 3 months of the onset of skin rash or muscle weakness.
Photos
- 69% of patients are willing to provide photos of signs and symptoms.
Month of First Symptom
- The incidence in every month is between 6% and 12%.
- Highest month is June at 12% and lowest month is July at 6%.
- The last 3 months of the year have the lowest incidence, aside from July.
Month of Diagnosis
- The month of diagnosis ranges between 6% (May) and 11% (October).
Length of Time Between Patient’s First Symptom and Diagnosis
- 27% were diagnosed between 1-3 months
- 27% were diagnosed between 4-6 months
Is the Patient in Remission (two years with no symptoms/no medications)?
- 78% of patients are NOT in remission

If Patient Went Into Remission, Did the Patient Have Any Relapses?
- 21% had relapses
- 23% did not have relapses
- 56% not applicable
Takeaway from this survey:
- ¼ of patients experience full remission
- ¼ of patients chronic with at least one remission
- ½ of patients chronic without remission
- Overall, ¾ of patients have chronic disease
(this is similar to Norwegian study that showed 61% of JDM patients had chronic disease after 16.8 years)
Medications Prescribed at Time of Diagnosis
- Top 3:
89% oral prednisone
62% methotrexate injections
58% IV solumedrol/prednisone
Most Important Outcomes Desired
- Top 3:
84% a cure
58% a “normal” lifestyle
20% better treatments
Types of Misdiagnoses Received Prior to JM Diagnosis
- Eczema 46%
- “Nothing is wrong” 32%
- Allergy 26%
- Flu or other virus 20%
- Psoriasis 13%
- Fifth’s Disease 11%
- Muscular Dystrophy 5%
The very FIRST symptom for the majority of patients
- Facial rash
- Non-specific rash
- Pain
- Fatigue/tiredness
- Rash on fingers, hands, knees, and/or elbows
The most DEBILITATING symptom for the majority of patients
- Muscle weakness
- Pain
- Fatigue/tiredness
- Skin involvement/rash
- Falling/difficulty walking
Geographic Area Where Child First Developed Symptoms
Top 5 States in the U.S.
- California
- Texas
- Illinois
- Florida
- New York

Top 5 Countries outside of the U.S.
- Canada
- United Kingdom
- Australia
- New Zealand
- South Africa