Description

Wedderburn et al reported a scoring system for evaluating muscle biopsies from a patient with juvenile dermatomyositis. This consists of 4 domains and a visual analogus scale (VAS).


Patient selection: juvenile dermatomyositis

 

Parameters for the inflammatory domain:

(1) CD3+ endomysial infiltration

(2) CD3+ perimysial infiltration

(3) CD3+ perivascular infiltration

(4) CD68+ endomysial infiltration

(5) CD68+ perimysial infiltration

(6) CD68+ pervascular infiltration

 

Finding

Points

< 4 positive cells per hpf (20x objective)

0

>= 4 positive cells per hpf (20x objective)

1

1 cluster with 10+ positive cells

1

> 2 clusters with 10+ positive cells in entire biopsy

2

diffuse infiltration

2

 

subscore for inflammatory domain =

= SUM(points for all of the parameters)

 

Parameters of the vascular domain:

(1) capillary dropout on a stain for CD31: obvious and marked decrease in the density of the capillary network, not restricted to the areas of perifascicular atrophy

(2) arterial abnormality: mural thickening and/or endothelial swelling and/or transmural inflammation

(3) infarction: well-demarcated regional loss of muscle fiber nuclei and loss of normal cellular architecture

 

subscore for vascular domain =

= (number of items present)

 

Interpretation:

• minimum subscores: 0

• maximum inflammatory subscore: 12

• maximum number of vascular items: 3


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