From the authors:
C. Xiaoqian and colleagues comment on our recent editorial [1], in which we emphasised the potential role of “Velcro” crackles at auscultation for earlier diagnosis of idiopathic pulmonary fibrosis (IPF). They are right to underline that “velcro” crackles were formerly one of the minor criteria for the diagnosis of IPF in the absence of a lung biopsy, and to discuss the role of high-resolution computed tomography (HRCT) of the chest.
Although “velcro” crackles alone are undoubtedly not specific of IPF, their finding should prompt the clinician to perform further investigations including chest HRCT, contributing to the early diagnosis of IPF, whatever the symptoms or the context that initially motivated lung auscultation. We further consider that pulmonary auscultation should still be included in the initial steps of the diagnostic algorithm in patients with chronic dyspnoea, especially in those with progressive dyspnoea, as well as in patients with chronic dry cough.
The contribution of HRCT in diagnosing IPF is well established, especially in light of the recent international guidelines, which state that in patients without identifiable cause of interstitial lung disease, a HRCT pattern of usual interstitial pneumonia (UIP) with honeycombing is diagnostic of IPF, obviating the need of a lung biopsy [2]. However, such patients, in whom the definite diagnosis of IPF can be made without a lung biopsy, already have a well-established and irreversible disease. Honeycombing on imaging unfortunately reflects our current failure to detect IPF at an earlier stage, with a risk for patients with markedly altered lung function tests of being excluded from clinical trials and even of being denied pirfenidone therapy. Therefore, we strongly advocate that patients with clinically suspected IPF and a pattern of possible UIP at imaging be given the chance of an early diagnosis confirmed by lung biopsy, even in the case of only mild symptoms and preserved lung function, if the benefit/risk ratio of a biopsy is favourable. Observing the development of honeycombing at imaging and the decline in lung function tests in a given patient long known to have crackles at auscultation and interstitial changes at imaging merely demonstrates that diagnostic (and possibly therapeutic) decisions are long overdue in this patient.
Improving the ability of community physicians to acknowledge “velcro” crackles at lung auscultation and especially to initiate appropriate investigations may be the only key for the earlier diagnosis of IPF: let us have the chance to hear crackles (and to diagnose and possibly treat IPF) before we see honeycombing.
Footnotes
Statement of Interest
Statements of interest for V. Cottin and J-F. Cordier can be found at www.erj.ersjournals.com/site/misc/statements.xhtml
- ©ERS 2013