Chest
Volume 132, Issue 2, August 2007, Pages 637-650
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Recent Advances in Chest Medicine
Recent Advances in Idiopathic Pulmonary Fibrosis

https://doi.org/10.1378/chest.06-1927Get rights and content

Idiopathic pulmonary fibrosis (IPF) remains the most common of the idiopathic interstitial pneumonias and portends a poor prognosis. Significant strides have been made in the approach to diagnosis and in the ability to predict outcome in the last few years. Advances in high-resolution CT (HRCT) scanning have allowed an accurate diagnosis obviating the need for surgical biopsy in many patients. Furthermore, HRCT scanning may aid in determining prognosis and identifying disease progression. The appropriate use of the HRCT scan requires a multidisciplinary iterative approach incorporating all available data to reach a final diagnosis. However, there remains great heterogeneity in disease progression. Pulmonary hypertension and acute exacerbations of IPF negatively influence prognosis and are increasingly a target of therapy. There has been an increase in the number of well-designed clinical trials of IPF that have focused on more specific targets. While no cure has yet been found, each trial expands our understanding regarding the natural course of the disease and the impact of targeted therapy. In the interim, lung transplantation, which appears to improve survival in a subset of IPF patients, remains the only intervention. The objective of this article is to review advances in the understanding of IPF and the evidence for the findings outlined above.

Section snippets

Diagnostic Issues

It is important to accurately diagnose IPF given the prognostic differences among the various idiopathic interstitial pneumonias. There is a growing body of literature addressing the diagnostic approach to these patients. Pivotal to the diagnoses of idiopathic interstitial pneumonias are refinements in the roles of high-resolution CT (HRCT) scanning and surgical lung biopsy (SLB).

Does HRCT Scanning Aid in Diagnosis?

HRCT scanning has improved the ability to make a definitive diagnosis without performing a biopsy in patients with some DPLDs, particularly IPF. Furthermore, the HRCT scanning aids in determining prognosis and may aid in identifying disease progression. As such, the community at large has adopted its use more definitively than ever before.6 But some caution must be exercised, as breadth of experience seems to matter.

A chest radiographic study usually demonstrates honeycombing and reticular

What Is the Value of HRCT Scanning in Defining Prognosis in IPF?

The extent and pattern of fibrosis on HRCT scans carry prognostic implications. For example, a multivariate analysis of a large multicenter trial24 suggested that a higher extent of fibrosis on HRCT scans was an independent predictor of mortality. When using a visual scoring method, IPF exhibits a progressive deterioration both in the extent of disease seen on HRCT scans and in lung function impairment over time, although the changes were subtle and evolved gradually. In clinical practice, the

Utility of SLB in IPF

Given the diagnostic limitations of imaging and the prognostic importance of a UIP diagnosis, lung biopsy continues to play an important role in the evaluation of a patient with DPLD.

Specific Clinical Situations Affecting Prognosis

As information continues to accrue, it has become clearer that a marked heterogeneity in disease progression exists in IPF patients. Many investigative groups have searched for indicators of disease progression. Among these are acute exacerbations of IPF and the development of pulmonary hypertension.

Pulmonary Arterial Hypertension

Pulmonary arterial hypertension (PAH) is a well-recognized sequela in patients with collagen vascular-associated DPLDs and in patients with DPLDs in general. The role of PAH in IPF is slowly being elucidated.

Therapeutic Approaches

The therapeutic approach to IPF is rapidly evolving, as reviewed by others.5758 In large part, the evolving nature of therapeutic approaches reflects the evolution of concepts regarding the biological processes that result in IPF.596061

Conclusions

The last few years have seen measurable advancements in the approach to diagnosis and defining prognosis in IPF patients. The diagnosis of IPF involves integration of the clinical history, and radiology and pathology findings. Within this triad, typical HRCT scan findings in the presence of a consistent clinical picture can allow for a confident diagnosis in the absence of a lung biopsy. The HRCT scan appearance may also provide prognostic information; an IPF patient with typical HRCT scan

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    This research was supported in part by National Institutes of Health, National Heart, Lung, and Blood Institute grants P50HL-56402 and 2 K24 HL04212.

    Dr. Noth has received grants, honoraria, or advisory fees from Actelion, Boehringer-Ingelheim, Cotherix, Centocor, GenZyme, GlaxoSmithKline, Intermune, Novartis Pfizer, and Roche. Dr. Martinez has received grants, honoraria and advisory fees from GlaxoSmithKline. He has also received honoraria and advisory fees from Boehringer Ingleheim, Pfizer, Novartis, Dey, and Schering Plough. He is a consultant for Altana Pharma.

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