• Paolo Spagnolo and Wim Wuyts.
• aSection of Respiratory Diseases, Department of Cardiac, Thoracic and Vascular Sciences, University of Padova, Padova, Italy bUnit for Interstitial Lung Diseases, Department of Respiratory Medicine, University Hospitals Leuven, Leuven, Belgium.
• Curr Opin Pulm Med. 2017 Sep 1; 23 (5): 411-417.

Purpose Of ReviewThe purpose of this review is to provide an update on acute exacerbation of interstitial lung disease (ILD), with a focus on idiopathic pulmonary fibrosis (IPF), in the light of the recently revised definition of acute exacerbation-IPF. Strengths and limitations of the current definition of acute exacerbation-IPF are also discussed.Recent FindingsClinically, acute exacerbation-IPFs are highly relevant events with a mortality rate of approximately 50%. A 2016 working group on acute exacerbation-IPF has suggested the definition be widened to include any acute respiratory worsening with new widespread alveolar abnormality on high-resolution computed tomography of the chest not fully explained by cardiac failure or fluid overload. Management of acute exacerbation-IPF typically includes supportive care, high-dose corticosteroids and broad-spectrum antibiotics, despite the scarcity of data supporting the usefulness of these therapies. The effect of a number of novel therapeutic approaches is currently under investigation.SummaryAcute exacerbation-IPF has recently been redefined. A standardized definition, similar to that of other chronic respiratory diseases, will likely facilitate the performance of highly needed studies in acute exacerbation of both IPF and other ILDs.

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