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Review
. 2015 Jun;10(4):401-11.
doi: 10.1007/s11739-015-1204-x. Epub 2015 Feb 12.

Acute exacerbation of idiopathic pulmonary fibrosis: a clinical review

Fabrizio Luppi  1 , Stefania Cerri, Sofia Taddei, Giovanni Ferrara, Vincent Cottin
Affiliations
Review

Acute exacerbation of idiopathic pulmonary fibrosis: a clinical review

Fabrizio Luppi et al. Intern Emerg Med. 2015 Jun.

Abstract

Idiopathic pulmonary fibrosis (IPF) is a chronic and progressive fibrotic disease limited to the lung, with high variability in the course of disease from one patient to another. Patients with IPF may experience acute respiratory deteriorations; many of these acute declines are idiopathic and are termed acute exacerbations (AE) of IPF. In these cases, the exclusion of alternative causes of rapid deterioration, including heart failure, bilateral pneumonia or pulmonary embolism, is a challenging goal. AE may occur at any time during the course of IPF, although they are more common in patients with more progressive disease and gastroesophageal reflux. Surgical lung biopsy or even surgical procedures in organs other than the lungs may also trigger AE, mainly in rapidly progressive or advanced IPF. Current diagnostic criteria include the presence of new-onset ground glass opacities or airspace consolidation superimposed on an underlying usual interstitial pneumonia pattern seen on high-resolution computed tomography. The outcome is poor with a short-term mortality in excess of 50% despite therapy. Currently, there is no treatment with demonstrated efficacy for AE-IPF: empirical high-dose corticosteroid therapy is generally used, with or without immunosuppressive agents, with limited evidence. On the other hand, there is hope that new treatments to slow down progression of IPF will translate into a reduction of AE-IPF's occurrence. In conclusion, although significant progress in assessing disease severity in IPF has been made, AEs remain unpredictable and are associated with a high risk of death. Improvements in our understanding of the etiology, risk factors, clinical predictors and epidemiology are needed. It is the goal of clinical researchers in the field to provide respiratory physicians with evidence-based guidance to identify patients who may benefit from therapy for preventing or treating AE-IPF.

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Figures

Fig. 1
Fig. 1
High-resolution computed tomography of the chest in a patient with AE-IPF. Upper panel UIP pattern with basal subpleural reticulation, traction bronchiectasis and mild honeycombing; lower panel 2 months later, superimposed ground glass opacities are visible, with architectural distortion
Fig. 2
Fig. 2
Postmortem pathologic examination of the right lung in a patient who died from acute exacerbation of IPF. a Macroscopic examination, showing dense, liver-like aspect of the lung, with reticulation on the surface. b Microscopic view of the lower lobe showing chronic fibrosis with UIP pattern with honeycombing and cystic changes and diffuse alveolar damage with hyperplastic pneumocytes (H&E stain magnification ×40) (courtesy of Dr Giulio Rossi, Modena, Italy)
Fig. 3
Fig. 3
Proposed diagnostic algorithm for acute exacerbation of idiopathic pulmonary fibrosis. (Modified from [14])
See this image and copyright information in PMC

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References

    1. Raghu G, Collard HR, Egan JJ, et al. An official ATS/ERS/JRS/ALAT statement: idiopathic pulmonary fibrosis: evidence-based guidelines for diagnosis and management. Am J Respir Crit Care Med. 2011;183:788–824. doi: 10.1164/rccm.2009-040GL. - DOI - PMC - PubMed
    1. King TE, Jr, Pardo A, Selman M. Idiopathic pulmonary fibrosis. Lancet. 2011;378:1949–1961. doi: 10.1016/S0140-6736(11)60052-4. - DOI - PubMed
    1. Cottin V. Current approaches to the diagnosis and treatment of idiopathic pulmonary fibrosis in Europe: the AIR survey. Eur Respir Rev. 2014;23:225–230. doi: 10.1183/09059180.00001914. - DOI - PMC - PubMed
    1. Selman M, Carrillo G, Estrada A, et al. Accelerated variant of idiopathic pulmonary fibrosis: clinical behavior and gene expression pattern. PLoS One. 2007;2:e482. doi: 10.1371/journal.pone.0000482. - DOI - PMC - PubMed
    1. Collard HR, Moore BB, Flaherty KR, et al. Acute exacerbations of idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 2007;176:636–643. doi: 10.1164/rccm.200703-463PP. - DOI - PMC - PubMed

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