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Table 2 Characteristics of the 23 patients with deteriorating lung function*

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

Parameter (n)

Time of deterioration month

Baseline

Deterioration

Extent of deterioration

Absolute value†

Absolute value†

P value‡

% of predicted

%

 

FVC (n = 6)

14.3 (8 · 4–23)

3350 (3100–3810)

2745 (2330–2950)

−665 (−770; −590)

0.03

  

98 (85–112)

80 (64–94)

−20 (−22.6; −16.2)

 

FEV1 (n = 13)

12.8 (5.8–18.2)

2540 (2390–3300)

1990 (1740–2770)

−460 (−530; −400)

<0.001

  

90.1 (80; 96 · 0)

77.2 (67 · 9; 78 · 7)

−16.7 (−18.8; −15.8)

 

DLCO (n = 14)

11.7 (6.2–17.6)

6 (4.7-6.5)

4.82 (3.6-5.31)

−1.17 (−1 · 4; −0.95)

<0.001

  

64.3 (57–72)

50 (44–60)

−17.8 (−19 · 2; −16 · 3)

 

6-minute walk distance, m

 

505 (480–547)

529 (471–564)

0 (−36; +31.5)

0.71

PaO2, mm Hg

 

79 (75–88)

86 (79–90)

−1 (−6; +12)

0.38

SGRQ score§

 

21.8 (10 · 8–43 · 5)

16.1 (7.3–28.7)

−3.2 (−11.2; +2.1)

0.12

HRCT nodular score║

 

7.5 (6–10)

6.5 (5–10)

0 (0–0)

0.69

HRCT cystic score

 

6.5 (5–11)

7 (4–12)

0 (0–2)

0.18

  1. Definition of abbreviations: FVC forced vital capacity, FEV 1 forced expiratory volume in 1 second, DL CO diffusion capacity for carbon monoxide, PaO 2 the arterial partial oxygen pressure, SGRQ St George’s Respiratory Questionnaire, HRCT high-resolution computed tomography.
  2. *Results are expressed as the medians and interquartile ranges (in parentheses). Lung function deterioration was defined as a decrease of at least 15% in the FEV1, FVC and/or DLCO.
  3. †Absolute values are expressed in ml for FVC and FEV1 and in mmol/min/kPa for DLCO.
  4. ‡A paired t-test was used for the comparisons.
  5. §SGRQ was available for 22 patients. Values ranged from 0 to 100, with higher scores indicating worse functioning.
  6. ║The maximal values for the HRCT nodular and cystic scores were 18 and 24, respectively.