Chest
ReviewsIdiopathic Pulmonary Fibrosis: A Rational Clinical Approach
Section snippets
Natural History of the Disease
Idiopathic pulmonary fibrosis was first recognized in an acute, fulminant and fatal form by Hamman and Rich.1 It is now evident that this acute form of the disease is uncommon and IPF is, more commonly, a chronic “disease” with slowly progressive interstitial pulmonary fibrosis. Patients have exertional dyspnea and cough some months to several years before diagnosis, and thus, the typical patient with IPF presents for medical evaluation at the midstage of the disease. The diagnosis of IPF
Insights Obtained from Bronchoalveolar Lavage in IPF
The technique of bronchoalveolar lavage (BAL) has been a great research tool in retrieving cells and proteins from the lower respiratory tract and has significantly contributed to the understanding of the pathogenesis of interstitial lung disease. It has been well demonstrated that BAL can be performed safely and reliably by community pulmonologists, and accurate results can be obtained from the analysis of the specimen that is transported to a central laboratory.5, 6 However, the implications
Other Limitations in the Management of IPF
Nearly 10 percent of patients with interstitial pulmonary fibrosis are reported to have normal chest roentgenograms, and yet, functional impairment in such patients may be very severe.13 While CT scans and magnetic resonance imaging of the chest may indeed clarify the anatomic extent of the parenchymal involvement, the “activity” of the disease process is not ascertained by these tests. Unless further studies show good correlation with the activity of the underlying disease (alveolitis vs
Suggested Clinical Approach to IPF
Since no full agreement on the best approach to a given patient with suspected IPF can be reached among clinical and research investigators of IPF, the following algorithm (Tables 2, 3 and 4) seems logical and practical, pending future definitive studies on management of patients with this frustrating disease.
The patient is suspected to have IPF when he presents with progressive exertional dyspnea, cough, restrictive pulmonary function impairment, and a diffuse roentgenographic pattern
ACKNOWLEDGMENTS
The comments of Leonard Hudson, M.D., and H. Thomas Robertson, M.D., are greatly appreciated, as is the secretarial assistance of Cathy Greenbaum.
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