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mortality but not the magnitude of honeycombing on HRCT (Takahashi et al., 2000). SP-A
and SP-D levels in serum seem to be autonomous indicators of mortality (Barlo et al., 2009;
Kinder et al., 2009), and their inclusion to clinical indicators alone may enhance prediction of
1-year mortality (Kinder et al., 2009). Extracellular matrix remodelling is significant in
matrix metalloproteinases (MMPs) and seems to be increased in both blood and BAL fluid in
patients with IPF. In one study, MMP-3, -7, -8, and -9 levels in BAL fluid were enhanced in
patients who died early in the follow-up (McKeown et al., 2009). As exhibited by another
study the MMP-7 was negatively associated with FVC and DlCO, but a relation with
prognosis was not basically studied (Rosas et al., 2008). A significant role in inflammatory
cell migration was exhibited by CC-chemokines (CCLs), and several members are increased
in IPF. CCL-18, a CC chemokine in serum, released by the alveolar macrophages, was
currently exhibited to be a powerful and autonomous indicator of mortality (Prasse et al.,
2009). Increased CCL-2, -17, and -22 in BAL fluids may anticipate poor outcome (Shinoda et
al., 2009). Mesenchymal cell progenitors are fibrocytes that are involved in tissue repair and
fibrosis, and the circulating levels are increased in IPF and enhance subsequently during
acute aggravations (Moeller et al., 2009). Their levels do not associate with the disease
seriousness by lung function or radiologic scores but seems to be an autonomous indicator of
early mortality. BAL cell counts eventually may be helpful in anticipating mortality. The
BAL neutrophil percentage at baseline has been exhibited to be autonomous to anticipate 1-
year mortality, while the lymphocyte and eosinophil percentages had no relation with
mortality (Kinder et al., 2008).
2.2.12 Management
The main cause for morality and illness is caused by IPF and so it reflects the huge
not met up medical requirement (Spagnolo et al., 2015).Anyhow, over the previous five years,
there is a remarkable strides to ameliorate the effect on IPF on patients by advanced and
recent pharmaceutical treatments.
In spite of these advances, the surgical intervention by lung transfer (LTx) is left over
as the sole therapy which can potentially remove the root cause of IPF related dyspnea (Yet
the above treatment is used as a last option).
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