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Correspondence |

Genesis of Some Histologic, BAL, and Auscultatory Features of Idiopathic Pulmonary FibrosisResponse FREE TO VIEW

Jerome M. Reich, MD, FCCP
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Affiliations: Earl A. Chiles Research Institute, Portland, OR,  University of Chicago, Chicago, IL,  University of Michigan Health System, Ann Arbor, MI

Correspondence to: Jerome M. Reich, MD, FCCP, Earl A. Chiles Research Institute, 5251 NE Glisan, Building A, Portland, OR 97213-2967; e-mail: Reichje@dnamail.com



Chest. 2008;133(2):585-586. doi:10.1378/chest.07-2466
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I enjoyed reading this thoughtful review1 of idiopathic pulmonary fibrosis (IPF). I believe the nature of the anatomic distortion might be more clearly and accurately conveyed by appreciating that the traction-induced distension predominantly affects the most compliant structures. Hence, reflecting their thin walls and absent cartilaginous support, the term traction bronchiolectasis is preferred to traction bronchiectasis. Similarly, the term alveolectasis might be substituted for honeycomb cysts, the bronchiolar epithelial lining of which appears to represent a metaplastic change (bronchiolated alveoli).

Mucin is a glycoprotein, responsible for the viscid consistency of mucus. The cystic spaces often contain mucus, which is a mixture of mucin, water, inorganic salts, epithelial cells, and leukocytes. Abundant neutrophils are frequently entrapped in the mucus.2 Their recovery in BAL fluid conveyed the erroneous idea that IPF was a neutrophilic alveolitis. Neutrophils found in the alveolar septae in IPF patients are rare,2and, as noted in the seminal article of Selman et al,3 the inflammatory mechanism differs qualitatively from that of an alveolitis.

The profuse rales seen in IPF patients may be attributable to the retained secretions in the ectatic bronchioles. Their characteristic quality (“Velcro rales”) may be due to the loss of attenuation provided in healthy lungs by surrounding air-filled alveoli. High-pitched, end-inspiratory wheezing accompanying the profuse rales (ie, chirping rales) may aid in the differential diagnosis of diffuse parenchymal lung disease by directing attention to a bronchiolitic component as in, for example, extrinsic allergic alveolitis.

The author has reported to the ACCP that no significant conflicts of interest exist with any companies/organizations whose products or services may be discussed in this article.

The authors have reported to the ACCP that no significant conflicts of interest exist with any companies/organizations whose products or services may be discussed in this article.

This research was supported in part by National Institutes of Health, National Heart, Lung, and Blood Institute Grant No. P50HL-56402 (Dr. Martinez), HL080513–02 (Dr. Noth), and NHLBI 2 K24 HL04212 (Dr. Martinez).

Noth, I, Martinez, FJ (2007) Recent advances in idiopathic pulmonary fibrosis.Chest132,637-650. [PubMed] [CrossRef]
 
Reich, JM Preliminary report: the origin of lavage neutrophils in fibrosing alveolitis associated with systemic sclerosis [letter].Sarcoidosis Vasc Diffuse Lung Dis1997;14,189-190. [PubMed]
 
Selman, M, King, TE, Pardo, A, et al Idiopathic pulmonary fibrosis: prevailing and evolving hypotheses about is pathogenesis and implications for therapy.Ann Intern Med2001;134,136-151. [PubMed]
 
To the Editor:

We believe that the authors of the letter regarding our article titled “Recent Advances in Idiopathic Pulmonary Fibrosis” that was published in a recent issue of CHEST (August 2007)1are insightful and raise numerous interesting points. The first issue involves nomenclature. The authors are correct to highlight the anatomic implications of current terminology and suggest alternative descriptive terms. The relationship between anatomic features noted histologically and the relationship to the more macroscopic view of the radiologist continues to evolve. The evolution of nosologic approaches to the interstitial pneumonias has produced quite confusing and contradictory literature. The attempts by international societies to standardize nomenclature23 and even to ensure application across practitioners45 have been a major advance. As such, we have preferred to utilize terminology that has remained widely used and accepted, even in the most recent reviews67 of the radiologic features of the interstitial pneumonias. The authors highlight the evolution of terminology, as this field is ripe with an overabundance of terms and abbreviations that are not always agreed on. Getting everyone to speak the same language is obviously a laudable goal but difficult to attain. As a start, ensuring that we all agree on what interstitial pulmonary fibrosis or usual interstitial pneumonia actually are in clinical, radiologic, and pathologic circles is probably an important start.

References
Noth, I, Martinez, FJ Recent advances in idiopathic pulmonary fibrosis.Chest2007;132,637-650. [PubMed] [CrossRef]
 
American Thoracic Society, European Respiratory Society.. American Thoracic Society/European Respiratory Society international multidisciplinary consensus classification of the Idiopathic Interstitial Pneumonias.Am J Respir Crit Care Med2002;165,277-304. [PubMed]
 
American Thoracic Society.. Idiopathic pulmonary fibrosis: diagnosis and treatment; international consensus statement.Am J Respir Crit Care Med2000;161,646-664. [PubMed]
 
Flaherty, KR, King, TE, Jr, Raghu, G, et al Idiopathic interstitial pneumonia: what is the effect of a multidisciplinary approach to diagnosis?Am J Respir Crit Care Med2004;170,904-910. [PubMed]
 
Flaherty, KR, Andrei, AC, King, TE, Jr, et al Idiopathic interstitial pneumonia: do community and academic physicians agree on diagnosis?Am J Respir Crit Care Med2007;175,1054-1060. [PubMed]
 
Müller-Mang, C, Stiebellehner, L, Schmid, K, et al Idiopathic interstitial pneumonias: from classification to diagnostic work-up.Radiologe2007;47,384-392. [PubMed]
 
Gotway, MB, Freemer, MM, King, TE, Jr Challenges in pulmonary fibrosis. 1: use of high resolution CT scanning of the lung for the evaluation of patients with idiopathic interstitial pneumonias.Thorax2007;62,546-553. [PubMed]
 

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References

Noth, I, Martinez, FJ (2007) Recent advances in idiopathic pulmonary fibrosis.Chest132,637-650. [PubMed] [CrossRef]
 
Reich, JM Preliminary report: the origin of lavage neutrophils in fibrosing alveolitis associated with systemic sclerosis [letter].Sarcoidosis Vasc Diffuse Lung Dis1997;14,189-190. [PubMed]
 
Selman, M, King, TE, Pardo, A, et al Idiopathic pulmonary fibrosis: prevailing and evolving hypotheses about is pathogenesis and implications for therapy.Ann Intern Med2001;134,136-151. [PubMed]
 
Noth, I, Martinez, FJ Recent advances in idiopathic pulmonary fibrosis.Chest2007;132,637-650. [PubMed] [CrossRef]
 
American Thoracic Society, European Respiratory Society.. American Thoracic Society/European Respiratory Society international multidisciplinary consensus classification of the Idiopathic Interstitial Pneumonias.Am J Respir Crit Care Med2002;165,277-304. [PubMed]
 
American Thoracic Society.. Idiopathic pulmonary fibrosis: diagnosis and treatment; international consensus statement.Am J Respir Crit Care Med2000;161,646-664. [PubMed]
 
Flaherty, KR, King, TE, Jr, Raghu, G, et al Idiopathic interstitial pneumonia: what is the effect of a multidisciplinary approach to diagnosis?Am J Respir Crit Care Med2004;170,904-910. [PubMed]
 
Flaherty, KR, Andrei, AC, King, TE, Jr, et al Idiopathic interstitial pneumonia: do community and academic physicians agree on diagnosis?Am J Respir Crit Care Med2007;175,1054-1060. [PubMed]
 
Müller-Mang, C, Stiebellehner, L, Schmid, K, et al Idiopathic interstitial pneumonias: from classification to diagnostic work-up.Radiologe2007;47,384-392. [PubMed]
 
Gotway, MB, Freemer, MM, King, TE, Jr Challenges in pulmonary fibrosis. 1: use of high resolution CT scanning of the lung for the evaluation of patients with idiopathic interstitial pneumonias.Thorax2007;62,546-553. [PubMed]
 
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