Figures
- FIGURE 1
Study design. IPF: idiopathic pulmonary fibrosis; AE-IPF: acute exacerbation of IPF.
- FIGURE 2
Prevalence of explanted lung pathology patterns in patients a) without (n=52) and b) with (n=28) acute exacerbation of idiopathic pulmonary fibrosis (AE-IPF). UIP: usual interstitial pneumonia; COP: cryptogenic organising pneumonia; DIP: desquamative interstitial pneumonia; DAD: diffuse alveolar damage.
Tables
- TABLE 1
Baseline characteristics and pre-evaluation data at time of lung transplantation listing for patients without acute exacerbation of idiopathic pulmonary fibrosis (AE-IPF) before lung transplantation and patients with AE-IPF before lung transplantation
No AE-IPF group AE-IPF group p-value Subjects 52 37 Baseline characteristics Age 66±7 67±5 0.42 Male/female 39/13 22/15 0.17 BMI kg·m−2 28±4 28±4 0.28 Comorbidities and smoking history Diabetes mellitus 11 (21) 12 (32) 0.33 Hypertension 18 (35) 21 (57) 0.051 Coronary artery disease 25 (48) 22 (59) 0.39 Congestive heart failure (systolic) 0 1 (3) 0.42 Smoking history 38 (73) 25 (68) 0.65 Ever-smoker pack-years 23±11 16±29 0.42 Pulmonary function tests FEV1/FVC 84±7 86±8 0.17 FEV1 % pred 52±15 50±15 0.42 FVC % pred 47±13 43±12 0.2 TLC % pred 48±9 46±9 0.41 RV % pred 44±15 33±10 0.004 DLCO % pred 28±11 24±10 0.17 Baseline oxygen saturation % 90±6 88±6 0.09 6-min walk distance m 254±99 218±74 0.08 Lowest oxygen saturation % 83±13 81±6 0.61 Blood tests Creatinine mg·dL−1 0.91±0.2 0.82±0.3 0.096 Albumin g·dL−1 3.4±0.53 2.95±0.54 0.0002¶ WBC cells·mm−3 12.8±6.5 14±5 0.23 Platelets cells·mm−3 222±71 212±113 0.63 PCO2 mmHg 43±9 44±11 0.54 Right heart catheterisation data mPAP mmHg 23±8 27±11 0.028¶ PCWP mmHg 8±4 7.5±5 0.46 Cardiac index L·min−1·m−2 3±2.7 2.7±0.7 0.53 PVR WU 3.4±2.6 4.4±2.9 0.11 Oesophageal pH and manometry data Number of total reflux episodes# 36±25 30±26 0.34 Time with pH <4# % 16±25 17±24 0.96 GORD score# 10±15 9±13 0.76 Data are presented as n, mean±sd or n (%), unless otherwise stated. BMI: body mass index; FEV1: forced expiratory volume in 1 s; FVC: forced vital capacity; TLC: total lung capacity; RV: residual volume; DLCO: diffusing capacity of the lung for carbon monoxide; WBC: white blood cells; PCO2: partial pressure of CO2; mPAP: mean pulmonary artery pressure; PCWP: pulmonary capillary wedge pressure; PVR: pulmonary vascular resistance; WU: Wood units; GORD: gastro-oesophageal reflux disease. #: based on 24-h oesophageal multichannel intraluminal impedance and pH monitoring: eight patients from the no AE-IPF and 10 patients from the AE-IPF groups did not complete the studies and were excluded from the GORD data analysis; ¶: not statistically significant when excluding patients evaluated and listed during AE-IPF.
- TABLE 2
Absolute forced vital capacity (FVC) decline and hazard ratios (HR) for developing acute exacerbation of idiopathic pulmonary fibrosis (AE-IPF) based on FVC decline (compared to <5% absolute decline)
No AE-IPF AE-IPF AE-IPF HR (95% CI) p-value Subjects# 48 22 Absolute FVC decline <5% pred 26 (54) 4 (18) Reference 5–10% pred 12 (25) 5 (23) 3.2 (0.77–13.5) 0.11 10–15% pred 4 (8) 5 (23) 5.6 (1.33–23.8) 0.019 >15% pred 6 (13) 8 (34) 7.3 (1.91–27.6) 0.004 Data are presented as n or n (%), unless otherwise stated. #: data were not available for four and 15 patients from the no-AE-IPF and AE-IPF groups, respectively.
- TABLE 3
High-resolution computed tomography (HRCT) findings, timing, ground-glass opacities (GGO) and correlation to pathologic findings in both groups
No AE-IPF AE-IPF Subjects 52 37 Baseline HRCT UIP pattern (typical/probable/indeterminate) 33/14/5 25/9/3 GGO in chest HRCT at any time before lung transplantation 17/52 (33) 19/37 (51) GGO severity in chest HRCT at any time scored by thoracic radiologist (mild/moderate/severe#) 5/2/10 3/0/16 Had chest HRCT 30 days prior to lung transplantation 2/52 (4) 13/28¶ (46) Had GGO in chest HRCT 30 days prior to lung transplantation 1/2 (50) 9/13 (69) Had GGO in chest HRCT 30 days prior to lung transplantation and acute pathological process in explant lung 0/1 9/9 (100) Data are presented as n or n (%). AE-IPF: acute exacerbation of idiopathic pulmonary fibrosis; UIP: usual interstitial pneumonia. #: mild: focal/patchy involvement, moderate: neither mild nor severe, severe: bilateral extensive; ¶: nine out of 37 patients died before receiving lung transplantation.
- TABLE 4
Timing of listing, hospitalisation and lung transplantation or death for both groups
No AE-IPF AE-IPF Subjects n 52 37 Time from hospitalisation to lung transplantation# days 0.4±0.5 21.6±24 Time from listing to transplantation days 164+175 Listed during AE-IPF¶ 20±30.8
Listed before AE-IPF¶ 167±146Time from AE-IPF to death before getting lung transplantation (n=9) days NA 37.8±32.7 Time from lung transplantation to discharge days 15.9+14.7 31.9±28.6 AE-IPF: acute exacerbation of idiopathic pulmonary fibrosis; NA: not applicable. #: time from hospitalisation for AE-IPF to lung transplantation; ¶: n=14.
- TABLE 5
Microbiological data for patients with acute exacerbation of idiopathic pulmonary fibrosis before mechanical ventilation
Patients examined Positive results Sputum bacterial culture 18 4# (22) Sputum viral culture or PCR 17 1 (6) Data are presented as n or n (%). #: all four patients with positive bacterial cultures died before being transplanted.
Supplementary Material
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TABLE S1 Study definitions 00261-2019.tableS1
