Prognostic Score of Jazek et al for Cryptogenic Fibrosing Alveolitis

 

NOTE: Cryptogenic fibrosing alveolitis has been applied to several conditions. It appears to correlate with idiopathic pulmonary fibrosis (page 1038, Fishman).

 

Parameters correlating best with prognosis:

(1) mean pulmonary arterial pressure at rest

(2) vital capacity

(3) carbon monoxide diffusing capacity

(4) age of onset

 

Parameter	Finding	Points
mean pulmonary arterial pressure at rest	> 30 mm Hg	3
	<= 30 mm Hg	0
vital capacity	<= 60% of predicted	2
	> 60% of predicted	0
CO diffusing capacity	< 40% of predicted	2
	>= 40% of predicted	0
age of onset	> 30 years of age	1
	<= 30 years of age	0

(see Figures 1 and 2, pages 714-715)

 

where:

• Diffusing capacity is in mL of CO (at STPD) per minute per mm Hg.

• The description of scores at the top of page 716 says "1 point for dyspnea appearing at an age under 30 years", but in the abstract and in Figure 2 the worse prognosis is with onset at age > 30 years.

 

prognostic score =

= SUM(points for findings present)

 

Interpretation:

• minimum score: 0

• maximum score: 8

• The presence of an increased resting mean arterial pressure with pulmonary hypertension is the most important predictive variable for prognosis.

 

Prognostic Score	Prognostic Group	6 year Survival Rate
0 - 3	good	93%
4 - 6	intermediate	56%
7 - 8	poor	0%

 

Limitations:

• The score was determined in 1980 and antedates several modern techniques, so that the 6 years survival rates should be improved. However, the relative prognosis is probably still relevant.