Description

King et al describe a clinical-radiographic-physiologic (CRP) scoring system for patients with idiopathic pulmonary fibrosis (IPF). This is based on a previous score reported by Watters et al. The authors are from the University of California at San Francisco, National Jewish Medical and Research Center and University of Colorado.


 

Patient selection: idiopathic pulmonary fibrosis (IPF)

 

Parameters:

(1) age in years (clinical finding)

(2) smoking status (clinical finding)

(3) clubbing (clinical finding)

(4) ILO profusion category (on chest radiograph

(5) pulmonary hypertension (on chest radiography)

(6) total lung capacity as percent of predicted (physiologic)

(7) PaO2 at maximal exercise in mm Hg (physiologic)

Parameter

Finding

Points

age in years

< 40 years

0

 

40 to 44.9 years

3.2

 

45 to 49.9 years

6.4

 

50 to 54.9 years

9.6

 

55 to 59.9 years

12.8

 

60 to 64.9 years

16

 

65 to 69.9 years

19.2

 

70 to 74.9 years

22.4

 

>= 75 years

25.6

smoking status

current

0

 

former (not in past year)

10.2

 

never

13.6

clubbing

no

0

 

yes

10.7

ILO profusion

< 15

0

 

15 to 17.49

9.15

 

>= 17.5

18.30

pulmonary hypertension

no

0

 

yes

10.3

TLC

>= 80% of predicted

0

 

70 to 79.9%

2.75

 

60 to 69.9%

5.50

 

50 to 59.9%

8.25

 

< 50% of predicted

11.00

PaO2 during maximum exercise

>= 65 mm Hg

0

 

60 to 64.9

1.5

 

55 to 59.9

3.0

 

50 to 54.9

4.5

 

45 to 49.9

6.0

 

40 to 44.9

7.5

 

35 to 35.9

9.0

 

< 35 mm Hg

10.5

 

where:

• Point assignment is a multiple of the a single value in most categories (3.2 for age, 1.5 for PaO2, 2.75 for TLC, 9.15 for profusion).

• A current smoker has less risk than a never smoker, all else being equal.

 

total score =

= SUM(points for all 7 parameters)

 

Interpretation:

• minimum score: 0

• maximum score: 100

• The higher the score the worse the survival. A score < 25 has a good long term survival while a score > 66 shows a sharp drop off.

• The authors also developed an “abbreviated” score that did not use the PaO2 during maximum exercise (see page 1179 and Figure 6).

 

Total Score

Survival

10

relatively flat with 90% survival at 10 years (excellent)

20

slight downward slope with 70% survival at 10 years (good)

30

downward slope with 50% survival at 10 years (fair)

40

downward slope with 50% survival at 66 months (guarded)

50

downward slope with 50% survival at 28 months and 0% survival at 120 months (suboptimal)

60

drops off with 0% survival at 78 months, 50% survival at 14 months (poor)

70

drops off with 0% survival at 36 months (poor)

80

sharp drop with 0% survival at 16 months (extremely poor)

90

sharp drop with 0% survival at 10 months (extremely poor)

 


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