Modern approaches to acute exacerbation of idiopathic pulmonary fibrosis


DOI: https://dx.doi.org/10.18565/therapy.2019.6.127-138

Avdeev S.N.

1) I.M. Sechenov First Moscow State medical University of the Ministry of Healthcare of Russia; 2) Pulmonology research Institute of FMBA of Russia, Moscow
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 are 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. Intrahospital mortality of 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.
Keywords: antifibrotic therapy, exacerbation, idiopathic pulmonary fibrosis, prognosis
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About the Autors

Sergey N. Avdeev, MD, professor, corresponding member of RAS, head of the Department of pulmonology of I.M. Sechenov First Moscow State medical University of the Ministry of Healthcare of Russia, head of Clinical Department of Pulmonology research Institute of FMBA of Russia. Address: 105077, Moscow, 28 Orekhovy boulevard. Tel./fax: +7 (495) 395-63-93. E-mail: serg_avdeev@list.ru. https://orcid.org/0000-0002-5999-2150


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