Prognostic scoring systems for clinical course and survival in idiopathic pulmonary fibrosis

Table 1 Summary of characteristics and main results of the studies published on Multidimensional-scoring systems

Ref.	Type of study (number of patients)	Variables included in the model	Summary of results
Gay et al[76]	Prospective (38)	HRCT score	HRCT fibrotic score ≥ 2: 80% sensitive and 85% specific in predicting death (34 mo average follow-up). The CRP does not add predicting value
		Pathology fibrotic score
King et al[77]	Retrospective (91)	Age	Only prediction results of single variables are reported by the authors. No direct data about performance of the CRP are reported
		Smoking status
		Clubbing
		HRCT score
		HRCT score for PH
		TLC % pred
		PaO2 at max exercise
Mogulkoc et al[78]	Retrospective (95)	HRCT score	HRCT and DLCO% combined model: AUC 0.91; sensitivity 84%, specificity 82% in predicting 2 yr survival
		DLCO % pred
Wells et al[79]	Retrospective (212)	DLCO % pred	5 yr survival CPI regression coefficient: 0.092 (0.043, 0.141). P < 0.0005
		FVC % pred
		FEV1 % pred
du Bois et al[80]	Prospective (830)	Age	Combined scoring system AUC: 0.75. 1 yr survival
		Respiratory hospitalization
		FVC % pred
		24 wk in FVC % pred
Richards et al[29]	Prospective (241)	Gender	PCMI ≥ 330: AUC 0.74-0.84 in predicting survival. Average follow-up 1.8 yr
		FVC %pred
		DLCO %pred
		MMP-7
Mura et al[82]	Prospective (138)	MRCDS	ROSE > 2:HR 11.4, P < 0.0001; AUC 0.76; sensitivity 39%, specificity 100% in predicting survival. 3 yr follow-up
		6MWD % pred
		CPI
Ley et al[83]	Retrospective (558)	Gender	GAP: c-index 69.3. Stages I, II and III 1-yr mortality of 6%, 16%, and 39%, respectively
		Age
		FVC % pred
		DLCO % pred

HRCT: High resolution computerized tomography; CRP: Clinical-radiographic-physiologic; TLC: Total lung capacity; DLCO: Diffusing capacity for carbon monoxide; CPI: Composite physiologic index; PCMI: Personal clinical molecular mortality index; MMP: Matrix metalloproteinase; MRCDS: Medical research council dyspnea score; 6MWD: 6 min walking distance; ROSE: Risk stratificatiOn ScorE; HR: High-resolution; GAP: Gender, age, physiology; PaO₂: Pulmonary arterial oxygen tension; FVC: Forced vital capacity; FEV1: Forced expiratory volume in 1 s.

Table 2 Risk stratification Score[82]

Low risk (all of the following conditions)	Intermediate risk (1 or 2 of the following conditions)	High risk (all the following conditions)
MRCDS ≤ 3	MRCDS > 3	MRCDS > 3
6MWD > 72% predicted	6MWD 72% ≤ predicted	6MWD 72% ≤ predicted
CPI ≤ 41	CPI > 41	CPI > 41

MRCDS: Medical research council dyspnea score; 6MWD: 6 min walking distance; CPI: Composite physiologic index.

Table 3 Gender, age, physiology index[83]

Stage	I	II	III
Points	0-3	4-5	6-8
	Predictor		Points
	Gender
G	Female		0
	Male		1
	Age
A	≤ 60		0
	61-65		1
	≥ 65		3
	FVC% predicted
	> 75		0
	50-75		1
P	< 50		2
	DLCO% predicted
	> 55		0
	36-55		1
	≤ 35		2
	Cannot perform		3
	Total possible points		8

FVC: Forced vital capacity; DLCO: Diffusing capacity for carbon monoxide.