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Table 1 Characteristics of PLCH patients included in the study

From: Clarifying the relationship between pulmonary langerhans cell histiocytosis and Alpha 1 antitrypsin deficiency

Characteristics Diffuse cystic pattern n = 24 Nodulo-cystic pattern n = 26
Age, years, median, [IQR] 40 [31–46.75] 45 [34–47]
Female sex, n (%) 13 (54%) 17 (65%)
Histological diagnosis 13 (54%) 3 (12%)
Isolated PLCH, n (%) 15 (62.5%) 23 (88%)
MS LCH, n (%) 9 (37.5%) 3 (12%)
Time from PLCH diagnosis, years, median, [IQR] 1 [0–10] 0 [0–5]
Smoker, n (%) 15 (62.5%) 24 (92%)
Ex-smoker 9 (37.5%) 2 (8%)
Dyspnoea 18 (75%) 16 (62%)
NYHA II/III/IV 9/8/1 14/2
TLC, % of predicted 104 [92.75–123.75] 105 [95.25–123]
FVC, % of predicted 78 [69.25–87.25] 100 [84.25–114]
RV/TLC, % of predicted 148.5 [118.5–166.75] 111.5 [103–132.5]
FEV1, % of predicted 58.5 [39.5–69.5] 86.5 [76–100]
FEV1/FVC % 66 [48.25–72] 77 [69.25–80.75]
DLCO, % of predicted, n = 23 37 [28–56] 52 [41–63]
Restriction* (%) 0 0
Obstruction (%) 17 (71%) 7 (27%)
Air trapping (%) 17 (71%) 10 (38%)
CT cyst score, median, [IQR] 20 [16.5–22] N/A
Alpha-1 antitrypsin level (g/L) (mean, SD) 1.39 (± 0.37) 1.41 (± 0.21)
PiM allele frequency 94% 100%
PiS allele frequency 4% 0%
PiZ allele frequency 2% 0%
  1. *Restriction was defined as TLC < 80% of predicted; obstruction as FEV1/FVC < 70% and air trapping as RV/TLC > 120% of predicted
  2. PLCH, pulmonary Langerhans cell histiocytosis; AAT, alpha-1 antitrypsin; IQR, interquartile range; NYHA New York heart association, MS multisystem; TLC total lung capacity; FVC forced vital capacity; RV residual volume; FEV1 forced expiratory volume in 1 s; DLCO diffusing capacity of carbon monoxide; N/A, not applicable