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Table 3 Univariate analyses of the predictive factors (measured at inclusion) of lung function deterioration*

From: The natural history of adult pulmonary Langerhans cell histiocytosis: a prospective multicentre study

Characteristic

Deterioration (n = 23)

No deterioration (n = 35)

HR (95% CI)

P value

Demographic features

    

Age, yrs

41.1 ± 12.0**

32.0 ± 8.2

1.7 (1.2-2.4)†

0.002

Sex, n (%)

    

 Male

12 (52)

15 (43)

1.0

 

 Female

11 (48)

20 (57)

0.98 (0.4-2.3)

0.97

Smoking status, n (%)

    

 Smokers

20 (87)

19 (54)

1.0

 

 Non-smokers

3 (13)

16 (46)

0.25 (0.1-0.85)

0.027

Clinical features, n (%)

    

 Asymptomatic

6 (26)

15 (43)

0.6 (0.2-1.7)

0.38

 Cough

10 (48)

10 (34)

1.3 (0.5-3.2)

0.54

 Dyspnoea

13 (57)

12 (34)

2.0 (0.8-4.6)

0.11

Lung function parameters

    

 FEV1

    

 % predicted

85.9 ± 13.7

87.8 ± 20.4

0.9 (0.7-1.2)†

0.45

 ≤59% predicted, n (%)

5 (14)

1 (4)

1 · 0

 

60-79% of predicted

7 (20)

6 (26)

2.8 (0.3-23.3)

0.35

 ≥80% predicted

23 (66)

16 (70)

2.6 (0.3-19.7)

0.36

 FVC

    

 % predicted

92.4 ± 19.6

93.4 ± 17.1

0.9 (0.7-1.2)†

0.92

 TLC

    

 % predicted

101.0 ± 18.0

100.3 ± 13.4

1.0 (0.8-1.3)†

0.98

 Restriction, n (%)‡

3 (13)

2 (6)

1.9 (0.5-6.4)

0.32

 RV

    

 % predicted

118.8 ± 33.4

115.0 ± 38.4

1.0 (0.9-1.1)†

0.67

 RV/TLC

36.1 ± 9.6

32.0 ± 9.6

1.0 (1.0-1.02)

0.58

 Air trapping, n (%)‡

10 (43)

11 (32)

1.7 (0.7-3.9)

0.23

 FEV1 /FVC

74.1 ± 9.8

76.5 ± 8.1

0.98 (0.9-1.0)

0.42

 Airflow obstruction, n (%)‡

10 (43)

5 (14)

2.9 (1.3-6.8)

0.014

 DLCO

    

 % predicted

63.8 ± 12.9

64.6 ± 13.2

0.3 (0.0-9.3)†

0.50

 <72% predicted, n (%)

17 (74)

24 (69)

1.0

 

 ≥72% predicted

6 (26)

11 (31)

0.7 (0.3-1.7)

0.42

 6-Minute walk distance, m

    

 % of the predicted value

78.7 ± 14.0

73.2 ± 10.1

1.4 (0.9-2.0)†

0.09

 PaO2

81.4 ± 9.7

90.8 ± 9.0

0.94 (0.91-0.98)†

0.0014

Lung HRCT§

    

 Nodular score

8.4 ± 4.8

7.8 ± 4.3

1.1 (0.7-2.0)║

0.46

 Cystic score

8.0 ± 3.9

8.4 ± 5.7

1.0 (0.7-1.4)║

0.89

SGRQ score

25.7 ± 20.1

15.1 ± 16.8

1.3 (1.1-1.6)†

0.012

  1. Definition of abbreviations: HR hazard ratio, CI confidence interval, FEV 1 forced expiratory volume in 1 second, FVC forced vital capacity, TLC total lung capacity, RV residual volume, DL CO diffusion capacity for carbon monoxide, PaO 2 arterial partial oxygen pressure, HRCT high-resolution computed tomography, SGRQ St George’s ;Respiratory Questionnaire.
  2. *Lung function deterioration was defined by a decrease of at least 15% in FEV1, FVC and/or DLCO compared with the baseline values.
  3. **Plus-minus values are the means ± SDs.
  4. †Reported HRs are given for an increase of 10 units.
  5. ‡Lung function restriction was defined as a TLC <80% of the predicted value, air trapping as an RV/TLC ratio >120% of the predicted value and obstruction as an FEV1/FVC ratio <70%.
  6. §HRCT was available at inclusion for 56 patients (22 and 34 patients in each group). The maximal values for the HRCT nodular and cystic scores were 18 and 24, respectively.
  7. ║Reported HRs are given for an increase of 4 points.
  8. ¶SGRQ was available for 55 patients at inclusion (23 and 32 patients in each group). The scores ranged from 0 to 100, with higher scores indicating worse functioning.
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Orphanet Journal of Rare Diseases

ISSN: 1750-1172