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Egyptian Journal of Chest Diseases and Tuberculosis (2015) 64, 915–919

H O S T E D BY
The Egyptian Society of Chest Diseases and Tuberculosis

Egyptian Journal of Chest Diseases and Tuberculosis


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ORIGINAL ARTICLE

Idiopathic pulmonary fibrosis (IPF) in upper Egypt,


a single center study
a,* b
M. Alaa Rashad , Ahmed K. Ibrahim

a
Department of Chest Diseases and Tuberculosis, Faculty of Medicine, Assiut University, Assiut, Egypt
b
Department of Public Health, Faculty of Medicine, Assiut University, Assiut, Egypt

Received 2 December 2014; accepted 10 May 2015


Available online 29 May 2015

KEYWORDS Abstract Aim of work: Idiopathic pulmonary fibrosis (IPF) is the second most common cause of
Interstitial pulmonary admission to Chest Department at Assiut University hospital, the pattern of presentation, method
fibrosis; for diagnosis and Co-morbidities need further studies.
Epidemiology; Aim: To explore demographic data and pattern of presentation of patients with IPF in upper
Upper Egypt Egypt and to study the difference from international data.
Methods: A total of 568 patients with final diagnosis of IPF were studied, retrospective study
was done using the available hospital database for all patients admitted at Assiut University hos-
pital (Tertiary hospital for all upper Egypt Governorates) during the period from 2007 to 2012.
Patients with incomplete data were excluded from study, all patients underwent chest X-ray, high
resolution computed tomography, spirometry, arterial blood gases, in addition to routine labora-
tory investigation. Patients with clinical or laboratory evidence of collagen vascular disease or
extrinsic allergic alveolitis were excluded from current study.
Results: The current study included 568 patients, 191 males and 377 females, mean age
44 ± 12 years. In all cases diagnosis was made according to clinical, spirometry and HRCT chest,
no one was subjected to thoracoscopic or bronchoscopic lung biopsy. Most cases were house wife or
farmer, 76% of cases were non-smokers, 17% ex-smokers and 7% current smokers, 86% of cases
have restrictive spirometry. Usual interstitial pneumonia was the most common high resolution
chest computed tomography pattern (51%) (Fig. 5). 39% of cases have at least one Co-morbid dis-
ease such as systemic hypertension, ischemic heart disease, pulmonary embolism and/or diabetes
mellitus.
There was a significant difference between male and female patients with IPF with regard to
smoking status (P value < 0.005) and HRCT pattern (P < 0.01).
Conclusion: IPF in upper Egypt has a different age and sex distribution compared to interna-
tional data. Domestic air pollution, indoor exposures or other environmental factors may account
for this difference. Lack of local resources for lung biopsy and lack of national guidelines for IPF

* Corresponding author.
Peer review under responsibility of The Egyptian Society of Chest
Diseases and Tuberculosis.
http://dx.doi.org/10.1016/j.ejcdt.2015.05.007
0422-7638 ª 2015 The Authors. Production and hosting by Elsevier B.V. on behalf of The Egyptian Society of Chest Diseases and Tuberculosis.
This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).
916 M. Alaa Rashad, A.K. Ibrahim

may also account for this difference. It is highly recommended to establish a national database for
patients with IPF in order to plane for national guidelines.
ª 2015 The Authors. Production and hosting by Elsevier B.V. on behalf of The Egyptian Society of Chest
Diseases and Tuberculosis. This is an open access article under the CC BY-NC-ND license (http://
creativecommons.org/licenses/by-nc-nd/4.0/).

Introduction generalizable to the present-day United States as a whole,


because they are based on data more than 15 yr old and come
Idiopathic pulmonary fibrosis (IPF) is a chronic progressive from an area of the United States known to attract persons
disease with unclear etiology and pathophysiology [1,2]. Its with chronic lung diseases [18].
prognosis is poor; survival time after initial diagnosis is only Although more recent estimates of disease prevalence
2.5–3.0 yr. There is currently no effective therapy known to (range, 1–24 cases per 100,000) are available from several
improve survival [3,4]. European studies, their generalizability to the United States
Numerous studies on the etiology of IPF have identified is not clear [19–23].
many possible inciting factors including saw dust, metal parti-
cles, smoking, gastroesophageal reflux, and viruses [1]. Since Methods
IPF is more prevalent in the aged population, it has been
hypothesized that the development and progression of IPF A single center retrospective hospital-based study was carried
may be affected by age-related diseases such as diabetes melli- out in chest department, Assiut University hospital, Assiut
tus (DM), metabolic syndrome, obesity, and cardiovascular Egypt (Tertiary hospital for all upper Egypt Governorates)
disease [5–8]. between January 2007 and December 2012.
Criteria for the diagnosis of IPF were set forth in an All patients with confirmed IPF admitted during the study
international consensus statement formulated by members period to Chest department at Assiut University, who agreed
of the American Thoracic Society (ATS) and the European to participate in the research, were included in this study.
Respiratory Society (ERS) [9]. In the absence of biopsy The diagnosis of IPF was made based on the diagnostic criteria
evidence of usual interstitial pneumonia, a constellation of of American Thoracic Society and the European Respiratory
typical clinical findings may be used to support the diagnosis Society by history taking, clinical examination, high-
of IPF. However, the extent to which these diagnostic resolution computerized tomography (HRCT) of the chest
guidelines are followed in actual clinical practice is and pulmonary function testing (PFT). None of the cases
unknown [9]. accepted to confirm the diagnosis by either thoracoscopic lung
Median survival among persons with IPF is believed to be biopsy or transbronchial lung biopsy. The presence of typical
from 3 to 5 yr. Respiratory failure is the most frequent cause of clinical and HRCT features of IPF, when identified by expert
death, and has been reported to account for over 80% [10,11– clinicians and radiologists, is sufficiently characteristic to allow
15]. a confident diagnosis and eliminate the need for surgical lung
Surprisingly, little is known about the epidemiology of IPF biopsy. All cases had basal fine crackles in auscultation and
in the United States. Initial estimates of its prevalence, based predominantly peripheral, sub-pleural, bi-basal fine reticular
largely on case series from pulmonary clinics and tertiary- shadows and/or honeycombing, occasionally with traction
care hospitals, ranged from 3 to 6 cases per 100,000 persons bronchiectasis on HRCT. All cases had also abnormal pul-
[16]. monary function studies including evidence of restriction––re-
Substantially higher rates of prevalence (20 per 100,000 duced vital capacity with increased FEV1/FVC ratio. There
among men, 13 per 100,000 among women) and incidence was no evidence of either coexisting collagen-vascular disease
(11 and 7 per 100,000, respectively) were reported in a study or history of known occupational exposure to agents that
based on the adult population of Bernalillo County, New might produce a clinical picture similar to that of IPF in any
Mexico [17]. It is unclear, however, whether these rates are of the cases.

Figure 1 Sex distribution. Figure 2 Occupation.


Idiopathic pulmonary fibrosis (IPF) in upper Egypt 917

The study was approved by the Research Ethics Committee


Assiut Faculty of Medicine, Assiut University.

Results

The current study included 568 patients, 191 males (34%) and
377 females (66%) (Fig. 1), mean age 44 ± 12 years. In all
cases diagnosis was made according to clinical, spirometry
and HRCT chest, no one was subjected to thoracoscopic or
bronchoscopic lung biopsy. Most cases were house wife 53%
or farmer 12% (Fig. 2), 76% of cases were non-smokers,
17% ex-smokers and 7% current smokers (Fig. 3), 86% of
cases have restrictive spirometry, mixed 9% or normal 5%
(Fig. 4). Usual interstitial pneumonia was the most common Figure 5 Chest high resolution Computed tomography pattern
high resolution chest computed tomography pattern (51%), (UIP = usual interstitial pneumonia, NSIP = non-specific inter-
desquamative 20% and nonspecific 5% (Fig. 5). 39% of cases stitial pneumonia).
have at least one Co-morbid disease such as systemic hyperten-
sion, ischemic heart disease, pulmonary embolism and/or dia-
betes mellitus (Fig. 6).
There was a significant difference between male and female
patients with IPF with regard to smoking status (P
value < 0.005) (Fig. 7) and HRCT pattern (Fig. 8) (P < 0.01).
Most females were non-smoker (97%).

Discussion

The current study included 568 IPF cases, showed that most
cases diagnosed with IPF in upper Egypt are middle aged

Figure 6 Co morbid Disease.

female house wife, epidemiology of IPF in the united states


showed that IPF is more prevalent among aged males.
In a population-based study in Bernalillo County, New
Mexico, Coultas et al. found a prevalence of ILD of 81 per
105 in males and 67 per 105 in females, and an incidence of
32 per 105 per yr in males and of 26 per 105 per yr in females
[24].
In another study, farming and raising birds with the poten-
tial exposures to dusts of animal feeds, products and waste as
well as pesticides were significant risk factors for the develop-
Figure 3 Smoking status. ment of IPF among female workers. Also, the environmental
exposure to domestic birds and cats was positively associated
with IPF development in both genders. These findings were
in accordance with results of current study.
Agricultural workers are exposed to very high levels of dust
and aerosolized particulates from a variety of sources includ-
ing feed grains, bedding, and livestock fecal material, [24]
and tend to have a higher prevalence of lung fibrosis [25].
In Egypt, the poultry industry had expanded rapidly over
the past 25 years to provide approximately 55% of the per cap-
ita animal protein consumption. Problems with raising birds in
Egypt include widespread roof-top and back-yard raising bird,
unhygienic local marketing and home slaughtering as well as
the presence of approximately 40,000 poultry farms lacking
biosecure and hygienic production systems and unprotected
exposure to birds. 30, 31 these widespread unplanned and
unprotected activities in raising birds and their environmental
Figure 4 Spirometry. impacts help in magnifying the role of raising birds in IPF
918 M. Alaa Rashad, A.K. Ibrahim

Figure 7 Smoking status according to sex distribution.

Figure 8 Chest HRCT pattern according to sex distribution. (UIP = usual interstitial pneumonia, NSIP = non-specific interstitial
pneumonia).

development. In Egypt, women were found to be more Physicians. Idiopathic pulmonary fibrosis: prevailing and
involved in raising birds than men and this may explain the ele- evolving hypotheses about its pathogenesis and implications
vated risk of IPF among women [24]. for therapy, Ann. Intern. Med. 134 (2001) 136–151.
This study has several limitations. First, data from the med- [2] S.W. Park, M.H. Ahn, H.K. Jang, A.S. Jang, D.J. Kim, E.S.
Koh, J.S. Park, S.T. Uh, Y.H. Kim, J.S. Park, et al, Interleukin-
ical records may be incomplete due to limited information.
13 and its receptors in idiopathic interstitial pneumonia: clinical
Second, this study was based on earlier, 2002 ATS-ERS guide- implications for lung function, J. Korean Med. Sci. 24 (2009)
lines for diagnosis of IPF and on retrospective study. 614–620.
Therefore, this study may have involved atypical IPF features [3] A.L. Olson, J.J. Swigris, D.C. Lezotte, J.M. Norris, C.G.
on HRCT. Third, the interpretation of HRCT images may Wilson, K.K. Brown, Mortality from pulmonary fibrosis
have differed in quality among radiologists. Further prospec- increased in the United States from 1992 to 2003, Am. J.
tive studies are needed to confirm our results using the new Respir. Crit. Care Med. 176 (2007) 277–284.
IPF guidelines. Fourth, the IPF in patients was not confirmed [4] S. Quadrelli, L. Molinari, L. Ciallella, J.C. Spina, E. Sobrino, J.
by lung tissue biopsy. Chertcoff, Radiological versus histopathological diagnosis of
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fibrosing alveolitis? A study of environmental exposure to
dust, BMJ 301 (1990) 1015–1017.
We have no conflict of interest to declare.
[6] R. Hubbard, S. Lewis, K. Richards, I. Johnston, J. Britton,
Occupational exposure to metal or wood dust and aetiology of
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