Figure 3. Hierarchical structure of 4 schemes represented as Venn diagrams.
(A) Griggs 1995 categories are nonoverlapping and nonhierarchical. (B) ENMC 2000 categories probable and definite are a nested hierarchy: all patients who meet definite criteria also meet probable criteria, and no gaps are present if a patient with probable gains additional features. (C) MRC 2010 categories are a hybrid between nested hierarchy (all CD patients meet Poss criteria) and nonoverlapping nonhierarchical (no PD patients meet CD or Poss criteria). This scheme contains a gap (both CD and Poss patients can gain an inclusion body myositis feature, such as tubulofilaments, resulting in loss of CD or Poss status, but yet not graduate to PD). (D) ENMC 2013 criteria are a hybrid between nested hierarchy (all CD patients meet Prob criteria) and an overlapping nonhierarchical category (CPD patients can also meet Prob or CD criteria, or they can fail to meet either Prob or CD criteria). CD = clinically defined; CPD = clinicopathologically defined; Def = definite; ENMC = European Neuromuscular Center; MRC = Medical Research Council; PD = pathologically defined; Poss = possible; Prob = probable.