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Abstract
ILDs are a heterogenous group of disorders with varying histologic appearance characterized by disruption of the pulmonary parenchyma [Schwartz, 1998].
ILDs has recently been reclassified as ”diffuse parenchymal lung disease” (DPLD) [ATS, 2002].
The most common form of ILDs seen in clinical setting is IPF, also called CFA, which accounts for approximately 25% to 35% of ILD cases [ATS/ERS, 2000].
Progressive interstitial destruction often leads to hypoxaemia due to an impairment of gas exchange, thus resulting in a high mortality rate [Kocheril et al., 2005].
The new gold standard for the diagnosis of IIP is a combination of clinical-radiographic-pathologic features arrived at by a dynamic, integrated process among clinicans, radiologists, and pathologists (when a surgical lung biopsy (SLB) is available) [BTS, 1999].
Plain chest x-rays are seldom helpful and a HRCT of the chest should be obtained whenever ILD is suspected [Orens et al., 1995].
HRCT has a central role in the diagnosis of diffuse lung disease in general and specially the IIP [Collard et al., 2003].
PFT is frequently used by the medical practioner in the management of patients with ILDs to aid in diagnosis, establish disease severity, define prognosis, and monitor response to therapy and disease progression [Johnston et al., 1997].
Smoking was shown to have a significant impact on both histopathology and lung function [Cherniack et al., 1995].
Exercise-induced hypoxia is one of the cardinal features of ILDs. This finding can be seen in early disease even in the absence of pulmonary function abnormalities [Risk et al.,1984].
BAL differential cell counts can provide important information that supports the diagnosis of specific ILDs [Meyer, 2004].
Fibreoptic bronchoscopy and TBB and lavage are of limited value in the diagnosis of IPF, but may be useful in identifying alternative or concomitant disorders such as opportunistic infection [BTS, 1999].
Not only can microarray genetic analysis provide characteristic gene expression pattern for specific ILDs, but distinct expression patterns may differentiate one ILD from another. Validation of this concept has been provided by Selman et al., [2006], who demonstrated distinct gene expression patterns in lung tissue for patients with IPF versus HP.
SLB is the method of choice for the definitive diagnosis of ILDs, and video-assisted thoracoscopic procedures have become the most favoured technique [Lettieri et al., 2005].
The ATS has published therapeutic guidlines for specific subtypes of ILDs [AT/ERS, 2000].
Although no therapy has ever been shown to be effective for IPF (60% of ILD cases), CS plus a steroid-sparing agent are often used as initial therapy if the patient desire treatment [ATS/ERS, 2000].
It was suggested that ealier treatment of AE-IPF with pulsed intravenous CS therapy might improve the outcome, based on a small clinical series [Suh et al., 2006].
Recently, there was a paradigm shift in the treatment of IPF, with more of a trend toward using antifibrotic treatment, based on the concept that the disease is a fibrotic process with a lack of significant inflammation [Zuo et al., 2002].
IFN therapy has been associated with development and worening of sarcoidosis [Pietropaoli et al.,1999].
IFN-γ was recently been shown to be ineffective in altering disease progression or mortality in a large multinational clinical trial [Raghu et al., 2004a].
Pirfenidone may improve lung function and reduce the number of episodes of AE-IPF [Azuma et al.,2005].
Acetylecysteine has recently been demonstrated to slow the progression of IPF in a randomized clinical trial combining N-acetylecysteine 600 mg p.o t.i.d with standard of care ( predinisone and azathioprine) [Demedts et al., 2005].
There is now accumulating evidence that treatment of PH in patients with ILD provides symptomatic benefit, may prolong survival, provides a bridge for patients awaiting transplantation, and may also modify the underlying ILDs [Maloney, 2003].
Some investigators have used CS successfully [Solomon and Anderson, 2001] to lower PAP.
Recent studies provided evidence that embryonic stem or adult stem cells can be used for cellular therapy in lung repair [Krause et al., 2001].
The only therapy shown to prolong life in patients with end-stage, particularly fibrotic ILDs is lung transplantation [International guidelines for the selection of lung transplant, 2006].