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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