Abstract
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. 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. 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. The Polish Respiratory Society Working Group developed guidelines for IPF diagnosis and treatment.
Highlights
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 a group of Polish experts
14 Should IPF patients with severe lung function impairment (FVC < 50% of predicted, TL,CO < 30% of predicted) be treated with antifibrotic agents? We suggest that antifibrotic treatment following IPF diagnosis be proposed to all patients without contraindications for this treatment, irrespective of the degree of lung function impairment
27 Should invasive ventilation be used in IPF patients We suggest that invasive ventilation NOT be used in IPF patients with acute respiratory failure? with acute respiratory failure
Summary
We suggest that a “probable UIP” HRCT pattern, if it contains no changes suggestive of an alternative diagnosis, in an appropriate clinical context (e.g. male sex, smoking history, age > 60) and after excluding other causes of such changes, should be a sufficient basis for a multidisciplinary team to diagnose IPF with no need for diagnostic lung biopsy. We recommend that a multidisciplinary team include at least a clinician (pulmonologist) and radiologist as well as pathologist (if lung biopsy has been performed). We suggest that patients with IPF diagnosis established by a multidisciplinary team based on the clinical context and the “probable UIP” pattern in lung HRCT be started on antifibrotic agents. We suggest that antifibrotic treatment following IPF diagnosis be proposed to all patients without contraindications for this treatment, irrespective of the degree of lung function impairment. 16 In what situations should one consider switching We suggest switching from one antifibrotic agent to the other in from one antifibrotic agent to the other?
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