Репозиторий Университета

Acute exacerbation of idiopathic pulmonary fibrosis

  • Avdeev S.
Дата публикации:01.01.2018
Журнал: Pulmonologiya
БД: Scopus
Ссылка: Scopus


© 2018 National Research University Higher School of Economics. All rights reserved. Acute exacerbation of idiopathic pulmonary fibrosis (AE-IPF) is defined as an acute clinically significant respiratory deterioration characterized by evidence of new, widespread alveolar abnormalities, such as diffuse bilateral ground-glass opacification and/or consolidation, and the absence of other obvious clinical causes like fluid overload, left heart failure, or pulmonary embolism, etc. AE-IPF is subcategorized as “triggered” (where specific causes are identified, for example, infections, surgery procedures, drug toxicity, etc.) or “idiopathic” (where no specific causes are identified). In randomized trials, the annual incidence of AE-IPF is about 8%, in retrospective studies it reaches 19%. Severe forms of IPF are an important risk factor for the development of AE-IPF. In-hospital mortality from AE-IPF is more than 50%, and the average survival of patients with AE-IPF is 1-4 months. Currently, there remain no proven, effective therapies for AE-IPF. In real clinical practice patients with AE-IPF still receive high doses of systemic corticosteroids and antibiotics. Antifibrotic therapy can reduce the risk of exacerbations; it has been shown that therapy with nintedanib leads to a reduction in the number of confirmed/suspected AE-IPF by 68%. It is necessary to further study the potential methods of prevention and therapy of AE-IPF in future clinical trials.

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