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cohort) of 14.6 per 100,000, while prevalence was 58.7 per 100,000 (Esposito et al., 2015).
The computed annual incidence/prevalence per 100,000 in Europe was 0.22/1.25 in Belgium,
0.94/6.5-12.1 in the Czech Republic, 0.93/3.38 in Greece, 2.17/NA in Denmark, 3.0/NA in
Spain, 4.3/23.4 in Norway, and 7.94/NA in the United Kingdom (Kuwano et al., 2016). A
study described the incidence/prevalence as 2.23/10.0 per 100,000 in Japan (Kuwano et al.,
2016).
The cause of IPF is unknown but is expected to be related with a variety of risk
factors, comprising of cigarette smoking, viral infections and occupation (Baumgartner,
2000). The most common collection of risk factors is exposure to inhalation agents, with
extended, repeated injury to the lungs substantially causing the fibrotic approaches (Kuwano
et al., 2016). IPF has also been perceived to appear in a familial pattern (influencing two or
more individuals of an immediate family), though the appearance of such familial cases
differs in studies between 2% and 25% of all IPF cases (Tang et al., 2003). One study
inference that among all the risk factors related with IPF, the highest single risk factor was to
have a parent or sibling with IPF (odds risk (OR) = 6.1) (García-Sancho et al., 2011).
Other than the genetic and environmental factors described above, there are numerous
medical conditions that incline to represent parallel to IPF. The 126 studies examined by
literature review conducted from 1990 to 2015, described remarkable comorbidities related
with IPF. The respiratory comorbidities were as follows: pulmonary hypertension (34%),
chronic obstructive pulmonary disease (18%), lung cancer (15%), obstructive sleep apnea
(6%), and pulmonary embolism (2%). The non-respiratory comorbidities comprised of
cardiovascular disease (27%), metabolic disease (24%), and gastro-esophageal reflux disease
(18%) (Raghu et al., 2015a). The category of these relations, whether they are causal or share
common risk factors (e.g., age), has not been described.
2.2.1 Idiopathic pulmonary fibrosis as an Interstitial Lung Disease
New international definitions, guidelines, classifications and treatment probabilities in
current years, have progressed in interstitial lung disease (ILD) and precisely in IPF. The
word ILD describes more than 200 different establishments. In 1960’s the first pathologic
categorization was defined, and in the subsequent 20-30 years no clear differentiation was
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