Summary
Introduction: This document presents the guidelines of the Polish Respiratory Society (PTChP, Polskie Towarzystwo Chorób Płuc) for diagnosis and treatment of idiopathic pulmonary fibrosis (IPF), developed by agroup of Polish experts.

Material and methods: The recommendations were developed in the form of answers to previously formulated questions concer-ning everyday diagnostic and therapeutic challenges. They were developed based on acurrent literature review using the Grading of Recommendations Assessment, Development and Evaluation (GRADE) methodology.

Results: We formulated 28 recommendations for diagnosis (8), pharmacological treatment (12) as well as non-pharma-cological and palliative therapy (8). The experts suggest that surgical lung biopsy (SLB) not be performed in patients with the probable usual interstitial pneumonia (UIP) pattern, with an appropriate clinical context and unanimous opinion of a multidisciplinary team. The experts recommend using antifibrotic agents in IPF patients and suggest their use irrespective of the degree of functional impairment. As regards non-pharmacological and palliative treatment, strong re-commendations were formulated regarding pulmonary rehabilitation, oxygen therapy (in patients with chronic respiratory failure), preventive vaccinations as well as referring IPF patients to transplant centres. Table 1 presents an aggregate list of recommendations.

Conclusions: The Polish Respiratory Society Working Group developed guidelines for IPF diagnosis and treatment.

Relevance
Idiopathic pulmonary fibrosis (IPF) is a chronic interstitial lung disease (ILD) limited to the lungs, associated with progressive fibrosis and consequentially with disturbances in diffusion of respiratory gases through the alveolar-capillary membrane, most commonly restrictive ventilation impairment, respiratory failure and premature death. The disease affects the elderly, more frequently males, and individuals with a history of tobacco smoking.

At present, the quantity and quality of evidence should be considered sufficient to recommend pulmonary rehabilitation in patients with IPF as an important part of treatment. The lack of conclusive data supporting a long-term effect indicates that rehabilitation should be applied continuously and systematically.

It is recommended for patients on long-term oxygen therapy who continue to be outdoors, as well as for patients participating in pulmonary rehabilitation programmes who have demonstrated improved physical performance as a result of a portable oxygen source. The results of the AmbOx study suggest that, compared with no oxygen therapy, the use of portable oxygen during exercise reduces desaturation and dyspnoea during exercise, and improves quality of life in patients with interstitial lung disease without resting hypoxaemia.