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ENMC IBM research diagnostic criteria 2011

ENMC IBM research diagnostic criteria 2011
Classification Clinical and laboratory features Pathological features
  Required for all:
  • Duration >12 months
  • Age at onset >45 years
  • Serum CK no greater than 15 times ULN
  • Endomysial inflammatory infiltrate
  • Rimmed vacuoles
  • Protein accumulation* or 15 to 18 nm filaments
Clinico-pathologically defined IBM

Knee extension weakness ≥ hip flexion weakness

and/or

Finger flexion weakness > shoulder abduction weakness		 All of the above features
Clinically defined IBM

Knee extension weakness ≥ hip flexion weakness

and

Finger flexion weakness > shoulder abduction weakness		 1 or more of above features
Probable IBM

Knee extension weakness ≥ hip flexion weakness

or

Finger flexion weakness > shoulder abduction weakness		 1 or more of above features

CK: creatine kinase; ENMC: European Neuromuscular Centre; IBM: inclusion body myositis; ULN: upper limit of normal.

* Demonstration of amyloid or other protein accumulation by established methods (eg, for amyloid Congo red, crystal violet, thioflavin T/S; for other proteins p62, SMI-31, TDP-43). Current evidence favors p62 in terms of sensitivity and specificity, but the literature is limited and further work is required.
Original figure modified for this publication. Rose MR, ENMC IBM Working Group. 188th ENMC International Workshop: Inclusion body myositis, 2-4 December 2011, Naarden, the Netherlands. Neuromuscul Disord 2013; 23:1044. Table used with the permission of Elsevier Inc. All rights reserved.
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