Classification of Juvenile Myositis
Classification of Juvenile Myositis
Lisa G. Rider, M.D. Environmental Autoimmunity Group National Institute of Environmental Health Sciences, NIH, DHHS
Bethesda, MD Email: RIDER@niehs.
Phone: 301-451-6272
1
IIM - Adult and Juvenile - Classifications
Clinical groups
Serologic groups
Dermatomyositis Polymyositis Myositis with other CTD Inclusion body Cancer-associated Focal / Nodular Ocular / Orbital
Myositis-specific (MSA) - Anti-synthetases - Anti-SRP - Anti-Mi-2
Myositis-associated (MAA) - Anti-U-RNP (U1, U2, U5) - Anti-Ro52 - Anti-PM/Scl
Eosinophilic
- Anti-Ku
Granulomatous
- Anti-155kD - Anti-MJ
Rider and Miller, Rheum Dis Clin N Amer,
- Anti-PMS-1, PMS-2
1997: 23: 619-655
MSA and MAA negative 2
Bohan and Peter are Primary Criteria Used in JDM Research Studies
Study Type
Clinical/ natural history/ epidemiol
Pathology/ pathogenesis
Immunogenetics
Therapeutic
Definite B&P 3*
4*
2 6
Probable B&P 31
4
9 7
Possible Not B&P defined
12**
5***
1
4
3 6
3
Bohan and Peter are Primary Criteria for Including JPM, JCTM in Research Studies
Study Type
Definite Probable
B&P
B&P
Clinical / natural
1
11
history/epidemiol
Pathology/
2
pathogenesis
Immunogenetics
3
Therapeutic
1
Possible B&P 3*
Not defined
3
2
4
Bohan and Peter Criteria are Often Present at Time of Diagnosis of JIIM
Criteria Rash
Positive % (n) Pachman, JDM
100% (79)
Positive % (n) Feldman, JIIM
94% (120)
Proximal weakness 100% (79)
92% (120)
Any 1 enzyme
CK
90% (79)
64% (76)
80% (120)
LDH
80% (56)
EMG
81% (43)
83% (104)
Muscle biopsy
80% (51)
92% (80) 5
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