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Table 4 Mean neutrality (sensitivity, specificity) of clinical studies measured against disease-specific disease severity scales

From: Neutral theory: applicability and neutrality of clinical study endpoints where a disease-specific instrument is available

Rare disease

Number of papers

Neutrality

Most Neutral Study

Least Neutral Study

Achalasia

27

2.00 (1.00, 1.00)

0.86 (0.00, 0.86)

Behcet’s disease

21

1.10 (0.10, 1.00)

0.61 (0.00, 0.61)

Creutzfeldt-Jakob disease

5

0.87 (0.12, 0.75)

0.75 (0.00, 0.75)

Fournier’s gangrene

5

2.00 (1.00, 1.00)

0.45 (0.00, 0.45)

Guillain–Barre syndrome

6

1.80 (0.91, 0.89)

0.33 (0.00, 0.33)

Atypical hemolytic uremic syndrome

6

0.71 (0.08, 0.62)

0.44 (0.00, 0.44)

Palmoplantar pustulosis

5

2.00 (1.00, 1.00)

0.00 (0.00, 0.00)

Prader-Willi syndrome

8

1.02 (0.11, 091)

0.49 (0.04, 0.45)

Systemic lupus erythematosus

22

2.00 (1.00, 1.00)

0.83 (0.04, 0,79)

Systemic sclerosis

28

2.00 (1.00, 1.00)

0.82 (0.00, 0.82)