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Basis of Acute Exacerbation of Idiopathic Pulmonary Fibrosis in Japanese Patients

We thank Dr. Wuyts and colleagues and Dr. Azuma and colleagues for their interest in our pulmonary perspective on acute exacerbations of idiopathic pulmonary fibrosis (IPF) (1). It was our hope in writing this perspective to stimulate thoughtful discussion, debate, and research on this important topic.

Dr. Wuyts and coworkers present an interesting algorithm for the diagnosis and management of acute exacerbations of IPF, and we agree with the authors' emphasis on aggressively looking for alternative etiologies (e.g., infection, pulmonary embolism). While the proposed approach to the diagnosis and management of acute exacerbations of IPF is a reasonable one, it is important to note that data supporting its specific recommendations are lacking. This is particularly true for the management recommendations, including the use of antibiotic, antifungal, and corticosteroid therapies. The authors' comments regarding the appropriateness of early referral for lung transplantation evaluation are strongly endorsed, as many patients with IPF may suffer acute worsening of their disease and death despite previously preserved pulmonary function (2, 3).

Nothing is known about susceptibility to acute exacerbation of IPF, but we agree with Dr. Azuma and coworkers that the Japanese preponderance of published cases may suggest an important genetic predisposition (4, 5). It is interesting to note that genetic and ethnic disparities exist in acute lung injury, a condition clinically and histopathologically similar to acute exacerbation of IPF (6). Better epidemiological data are sorely needed to further investigate Dr. Azuma's hypothesis.

Harold R. Collard

University of California San Francisco
San Francisco, California

Fernando J. Martinez

University of Michigan
Ann Arbor, Michigan

FOOTNOTES

Conflict of Interest Statement: H.R.C. has served as a consultant for Intermune, Genzyme, and Gilead. F.J.M. has no financial relationship with a commercial entity that has an interest in the subject of this manuscript.

REFERENCES

1. Collard HR, Moore BB, Flaherty KR, Brown KK, Kaner RJ, King TE Jr, Lasky JA, Loyd JE, Noth I, Olman MA, et al.; Idiopathic Pulmonary Fibrosis Clinical Research Network Investigators. Acute exacerbations of idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 2007;176:636–643.[Abstract/Free Full Text]
2. King TE Jr, Safrin S, Starko KM, Brown KK, Noble PW, Raghu G, Schwartz DA. Analyses of efficacy end points in a controlled trial of interferon-1b for idiopathic pulmonary fibrosis. Chest 2005;127:171–177.[CrossRef][Medline]
3. Martinez FJ, Safrin S, Weycker D, Starko KM, Bradford WZ, King TE Jr, Flaherty KR, Schwartz DA, Noble PW, Raghu G, et al. The clinical course of patients with idiopathic pulmonary fibrosis. Ann Intern Med 2005;142:963–967.[Abstract/Free Full Text]
4. Kondo A, Saiki S. Acute exacerbation in idiopathic interstitial pneumonia (IIP). In: Harasawa M, Fukuchi Y, Morinari H, editors. Interstitial pneumonia of unknown etiology. Japan Intractable Diseases Research Foundation Publication No. 27. Tokyo: University of Tokyo Press, 1989. pp. 33–42.
5. Kubo H, Nakayama K, Yanai M, Suzuki T, Yamaya M, Watanabe M, Sasaki H. Anticoagulant therapy for idiopathic pulmonary fibrosis. Chest 2005;128:1475–1482.[CrossRef][Medline]
6. Barnes KC. Genetic determinants and ethnic disparities in sepsis-associated acute lung injury. Proc Am Thorac Soc 2005;2:195–201.[Abstract/Free Full Text]

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