INTERSTITIAL LUNG DISEASES WITH PROGRESSIVE PULMONARY FIBROSIS (NON-IDIOPATHIC PULMONARY FIBROSIS). NEW INTERNATIONAL CLINICAL GUIDELINES

Abstract

Pulmonary fibrosis is not unique to idiopathic pulmonary fibrosis (IPF), although, for many years, treatment options were available only for this disease. The prognosis of the disease remains poor, but antifibrotic drugs have significantly prolonged the survivability of patients. In 2022 American Thoracic Society (ATS), European Respiratory Society (ERS), Japanese Respiratory Society (JRS) and Asociacion Latinoamericana de Torax (ALAT) clinical practice guidelines were supplemented with the term progressive pulmonary fibrosis (formerly known as progressive fibrosing interstitial lung disease) and recommendations on how to diagnose and treat interstitial lung diseases with progressive pulmonary fibrosis, but which do not meet the criteria of IPF. This article reviews guidelines that clearly distinguish between progressive pulmonary fibrosis and IPF, provides diagnostic criteria for progressive pulmonary fibrosis in interstitial lung disease (non-IPF), and reviews the current data on the efficacy and safety of antifibrotic drugs.