Exacerbation of idiopathic pulmonary fibrosis

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Abstract

Idiopathic pulmonary fibrosis (IPF) is usually characterized by a chronic and slowly progressive course. According to several studies, a small number of patients with IPF (about 5–15%) develops an acute deterioration of deasese – exacerbation of IPF. Exacerbations of IPF can occur at any time of the disease and sometimes becomes the first manifestation of IPF. Pulmonary hypertension in IPF is a fairly frequent complication, which leads to severe violations of gas exchange and reduced tolerance to physical stress. Currently, proven effective treatments for exacerbations of IPF do not exist, the management of this condition is based on supportive therapy (oxygen, respiratory support) and interventions with inadequate evidences (corticosteroids, immunosuppressant). During exacerbation of IPF a careful search of all the possible triggers is justified. In the presented clinical case of exacerbation of IPF there was demonstrated the efficacy of complex therapy including antifibrotic therapy, PAH-specific medicines and enhanced oxygen therapy.

About the authors

S. N. Avdeev

Sechenov First Moscow State Medical University (Sechenov University); Research Institute of Pulmonology

Email: ivv_08@mail.ru
ORCID iD: 0000-0002-5999-2150

чл.-кор. РАН, д.м.н., проф., зав. каф. пульмонологии лечебного фак-та

Russian Federation, Moscow

V. V. Gaynitdinova

Sechenov First Moscow State Medical University (Sechenov University)

Author for correspondence.
Email: ivv_08@mail.ru
ORCID iD: 0000-0001-9928-926X

д.м.н., проф. каф. пульмонологии лечебного фак-та

Russian Federation, Moscow

Z. M. Merzhoeva

Sechenov First Moscow State Medical University (Sechenov University); Research Institute of Pulmonology

Email: ivv_08@mail.ru
ORCID iD: 0000-0002-3174-5000

к.м.н., ассистент каф. пульмонологии лечебного фак-та

Russian Federation, Moscow

G. V. Neklyudova

Sechenov First Moscow State Medical University (Sechenov University); Research Institute of Pulmonology

Email: ivv_08@mail.ru
ORCID iD: 0000-0002-9509-0867

д.м.н., доц. каф. пульмонологии лечебного фак-та

Russian Federation, Moscow

N. A. Tsareva

Sechenov First Moscow State Medical University (Sechenov University); Research Institute of Pulmonology

Email: ivv_08@mail.ru
ORCID iD: 0000-0001-9357-4924

к.м.н., доц. каф. пульмонологии лечебного фак-та

Russian Federation, Moscow

G. S. Nuralieva

Sechenov First Moscow State Medical University (Sechenov University); Research Institute of Pulmonology

Email: ivv_08@mail.ru
ORCID iD: 0000-0002-4726-4906

к.м.н., доц. каф. пульмонологии лечебного фак-та

Russian Federation, Moscow

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