Lung Function in Combined Pulmonary Fibrosis
and Emphysema Syndrome, What to Expect
Jolanda Nikolla1*, Hasan Hafizi2, Roland Hasa3
1Department of Internal Medicine, American Hospital, Albania
2Department of Pulmonology, UH “Shefqet Ndroqi”, Albania
3Department of Radiology, UH “Shefqet Ndroqi”, Albania
Submission: June 07, 2017; Published: July 26, 2017
*Corresponding author: Jolanda Nikolla, Department of Internal Medicine, Pulmonologist, Subdivision Pulmonology, American Hospital 3, Tirana, Albania, Tel: +355 69 224731; Email: [email protected]
How to cite this article: Jolanda N, Hasan H, Roland H.Lung Function in Combined Pulmonary Fibrosis and Emphysema Syndrome, What to Expect. Int
002 J Pul & Res Sci. 2017; 2(1): 555578. DOI:10.19080/IJOPRS.2017.02.555578
Introduction: Combined Pulmonary Fibrosis and Emphysema Syndrome (CPFE) is a distinct entity and has been identified as such recently. In contrast with Dlco reduced lung function is preserved in these patients.
Objective: Our aimed was to study the differences in the lung function, demographic and clinical data of CPFE syndrome versus IPF patients.
Materials and Methods: We studied retrospectively the medical records of the patients with interstitial lung diseases during the time period 2012 - 2016. The diagnosis was based on HRCT findings based on ATS/ERS criteria the lung function, demographic and clinical data were collected. EVIEWS 7 and SPSS programs were used for statistical study. Mean values and standard deviation were used for normal variables. Student test and Pearson Correlation Coefficient were used for numerical variables and for evaluating the existence and their importance in correlations. P values less than 0.05 were statistically significant.
Results: In total 53 (100%) subjects with ILD.27 (50.9%) CPFE patients and 25 (49.1%). Compared to IPF in CPFE group pre-dominated male’s current smokers or ex smokers and the mean age was higher (67.29±6.87). Lung volumes were closer to normal values in CPFE patients. Dlco was lower in this group (47.9±16). PAH was more frequent too (42.8%). There was an important and positive statistical correlation between Dlco and CPI (r = - 0.7, p = 0.003).
Conclusion: CPFE syndrome is frequent. Its diagnosis should be earlier. There are differences in CPFE syndrome and IPF patients mainly in lung function parameters.
Keywords: Lung function; Combined pulmonary fibrosis and emphysema syndrome
Interstitial lung diseases (ILD) are a large and heterogeneous group of lung clinical entities. Among them idiopathic pulmonary fibrosis (IPF) is the most frequent . The coexistence of pulmonary emphysema and IPF in the same patient is recently recognized as a distinct syndrome titled “Combined Pulmonary Fibrosis and Emphysema” (CPFE), by Cottin et al. [1-3]. Smoking and connective tissue diseases are the most encountered etiologic factors causing IPF and CPFE syndrome. The clinical, functional and radiological status of these patients has important differences compared to them of IPF only . Demographics features of CPFE and IPF are nearly similar but smoking history
is longer and heavier in CPFE group. In particular there is distinction in lung function presentation [1-3]. CPFE patients have higher lung volumes (FEV1- forced expiratory volume of the first second, FVC- forced vital capacity, FEV1/FVC ratio in %) lower values of diffusion lung capacity for carbon monoxide (Dlco), more profound hypoxemia and lower values of oxygen saturation in arterial blood (Sat O2 %). Pulmonary arterial hypertension (PAH) also has higher prevalence in CPFE patients. It is encountered earlier in CPFE than IPF, however it represents a significant prognostic factor for mortality in both pathologies [1-5] (Figure 1).
This study is approved by Ethics Committees of the University
of Tirana, and the University Hospital “Shefqet Ndroqi”, in
Tirana, Albania where the work has been undertaken. This is a
retrospective cohort study of the time period 2012-2016.
53 patients in total were enrolled in the study. They were
all diagnosed with ILD based on American Thoracic Society/
European Respiratory Society (ATS/ERS) guidelines [6,7]. We
have collected the demographics, clinical, lung function and
high-resolution computed tomographic (HRCT) data of the
patients from their medical records. The HRCT scans findings
were evaluated from two different radiologists independently
from each other and without having any knowledge about the
clinical picture of the patients.
HRCT criteria for diagnosis of CPFE syndrome [3-7] were as follows:
The presence of bi-apical lung emphysema and/or
multiple bullae (> 1 cm diameter) AND
The presence of significant bilateral lung fibrosis with
peripheral and basal predominance, with/without traction
bronchiectasis and with/without honey combing.
After discussions we had selected the patients in two large
groups: 28 CPFE patients and 25 IPF patients.
The patients with the following characteristics were
excluded from the study:
Patients aged <50 years
Patients diagnosed with drug-associated ILD
Patient diagnosed with occupational related ILD
According to the etiologic factors  the two large groups
are divided further in subgroups as follows: CPFE group is
divided in: 1.Idiopathic CPFE (smoking) 2.CPFE in connective
tissue diseases (CPFE-CTD).IPF group is divided in: 1.Idiopathic
IPF (smoking) 2.IPF in connective tissue diseases (IPF-CTD).
Concerning smoking, patients are classified as: never smokers,
ex-smokers and current smokers. For evaluating smoking
status we have used Unit Pack Year formula: UPY = (number of
cigarettes smoked per day/20) x number of years smoked. For
evaluating dyspnea status we have used m MRC (the modified
Medical Research Council) score and the patients were classified
as having dyspnea one of scores I, II, III or IV . Dlco test was
performed from not all the patients due to the severe conditions
of them. The composite physiological index (CPI) was calculated
according to the formula: The extent of the fibrosis in CT = 91.0
- (0.65 x predicted of DLCO %) - (0.53 x predicted of FVC %)
+ (0.34 x % predicted of FEV1 %). The GAP (gender, age and
physiology) index and the mortality risk scoring system was
calculated too. PAH was diagnosed as having resting sPSAP ≥ 25
mm Hg or in effort sPSAP ≥ 30 mm Hg values [9-11].
For statistical analyses were used EVIEWS 7 and SPSS version
20 (SPSS-Statistical Package for Social Sciences Inc., Chicago,
IL, USA) programs. Demographics, clinical and lung functional
data are expressed in mean values and standard deviation ± SD.
For testing numerical and independent categorical variables
is used Student test. Pearson Correlation Coefficient is used
for numerical variables for evaluating the existence and their
importance in correlations. Hypotheses were tested using disfactorial
linear regression. Logistic binary regression was used
for evaluating the correlations between the variables. P values
less than 0.05 were accepted as statistically significant.
The percentage of CPFE in our group was high (50.9%).
Table 1 shows more specifically the distribution of the most
important demographics and clinical data of the subgroups. In
total 53 patients (100%). From them 45.3% idiopathic CPFE
patients, 41.5% idiopathic IPF, 7.5% CPFE-CTD and 5.7% IPFCTD.
The mean age of the patients was higher idiopathic CPFE
(67.29±6.87). Males predominated in three first subgroups,
70.8%, 54.5% and 50% respectively. Current smokers or exsmokers
males were also the most of the subgroups. UPY values
were higher in idiopathic CPFE (28.4±13.2) and CPFE-CTD
(23.7±13.8) subgroups. 50% of Idiopathic CPFE had mMRC III dyspnea score. Finger clubbing was more frequent in idiopathic
IPF subgroup (13.6%). Table 2 shows the lung function
parameters in subgroups. It was evident that CPFE subgroups
had lower mean values of predicted FVC % and FEV1 compared
to IPF subgroups.FEV1/FVC ratio % mean values tended to be
more close to the normal ranges (90±22.7) in idiopathic CPFE
subgroup. Mean values of Sat O2 % and PaO2 were lower in
idiopathic CPFE compared to other subgroups. Dlco mean
values were lower in idiopathic CPFE subgroup (47.9±16) and
the majority of the patient (83.4% idiopathic CPFE subgroup)
had high mortality rate in the second year of the diseases (Table
3). PAH was more frequent in the large group of CPFE (42.8%)
and mean sPSAP values were higher too in this group (42.4±3.4)
compared with the large group of IPF (39.6±4.7). Using Pearson
test were analyzed if there were any correlation of DLco with the
lung function variables such as CPI, FVC, FEV1, PaO2 and was also
evaluated the importance of Dlco especially in CPFE patients,
(Figure 2a-2e). There was an important and positive statistical
correlation between Dlco and CPI (r = - 0.7 p = 0.003), the other
variables did not have important correlations with Dlco, (Table 4).
The presence of emphysema in IPF patients (CPFE syndrome)
is 21-33% based in recently literature [1,2]. In our study it
was higher 50.9%. We had males’ smokers or ex-smokers with
high values of UPY and in older age too. Lung volumes in CPFE
patients were close to normal values. Dlco values were more
compromised in CPFE patients. Our group of CPFE patients had
higher values of sPAP as it is prescribed in published papers [11,12]. We did not find important correlations among Dlco and
the other lung function parameters in our study population. We
should also emphasize that our study had limitations such as the
relatively small number of the patients; it was a retrospective
study and the information was taken from medical records so
that some data might be lost. However lung function in our
group of CPFE had differences compared to IPF patients group.
Patients with CPFE syndrome have different lung function
picture than IPF patients but they are underestimated. Usually
they are grouped with IPF patients. We should be more prudent
on diagnosing earlier these patients given the fact that this
syndrome is not rare anymore.