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Acute exacerbation of IPF

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By Jason Mann No BS pulmonary critical care fellow
Christopher J Ryerson, Vincent Cottin, Kevin K Brown, Harold R Collard
The goal of this review is to summarise the clinical features, management, and prognosis of acute exacerbations of idiopathic pulmonary fibrosis (AE-IPF). AE-IPF has previously been defined based on clinical and radiological features that include the subacute onset of dyspnoea, bilateral ground glass changes on chest high-resolution computed tomography, and the absence of an identifiable aetiology. The annual incidence of AE-IPF is typically reported at 5-15%, but is less common in mild disease. Features of diffuse alveolar damage are present when a biopsy is performed...
August 2015: European Respiratory Journal: Official Journal of the European Society for Clinical Respiratory Physiology
Khuder Alagha, Veronique Secq, Laurie Pahus, Tunde Sofalvi, Alain Palot, Arnaud Bourdin, Pascal Chanez
No abstract text is available yet for this article.
April 15, 2015: American Journal of Respiratory and Critical Care Medicine
Maya M Juarez, Andrew L Chan, Andrew G Norris, Brian M Morrissey, Timothy E Albertson
Idiopathic pulmonary fibrosis (IPF) is a chronic and progressive form of lung disease of unknown etiology for which a paucity of therapies suggest benefit, and for which none have demonstrated improved survival. Acute exacerbation of IPF (AE-IPF) is defined as a sudden acceleration of the disease or an idiopathic acute injury superimposed on diseased lung that leads to a significant decline in lung function. An AE-IPF is associated with a mortality rate as high as 85% with mean survival periods of between 3 to 13 days...
March 2015: Journal of Thoracic Disease
Samy Suissa, Pierre Ernst
No abstract text is available yet for this article.
May 2015: Thorax
Hammad Bhatti, Ankur Girdhar, Faisal Usman, James Cury, Abubakr Bajwa
Idiopathic pulmonary fibrosis (IPF) is a chronic interstitial pneumonia with a median survival of 3 years after diagnosis. Acute exacerbation of IPF (AE-IPF) is now identified as a life-threatening complication. It presents as worsening dyspnea with new ground glass opacities superimposed upon a radiographic usual interstitial pneumonia (UIP) pattern. It is a diagnosis of exclusion. The prognosis of AE-IPF is poor and treatment strategies lack standardization. In order to rule out any reversible etiology for an acute decompensation of a previously stable IPF patient diagnostic modalities include computerized tomographic angiogram (CTA) coupled with high-resolution computerized tomography (HRCT) imaging of the chest, bronchoalveolar lavage (BAL) and echocardiogram with bubble study...
April 2013: Annals of Thoracic Medicine
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