Abstract
Patient management in Idiopathic Pulmonary Fibrosis (IPF) is largely based on societal guidelines and recommendations. A recent update by the American Thoracic Society (ATS), European Respiratory Society (ERS), Japanese Respiratory Society (JRS) and Latin American Thoracic Association (ALAT) provided updated guidance on the diagnosis and management of IPF, along with recommendations on pharmacologic and non-pharmacologic approaches to patient management. The treatment guidance is based on GRADE criteria, which rates the quality of evidence according to previously published methodology. Here we discuss how to interpret the recent guideline updates and the implications of this guidance for clinical practice. In addition we discuss the assessment and recommendations for a number of pharmacological agents that have been the focus of clinical trials over the past years. Although no single pharmacological agent was recommended by the guidelines committee, we discuss how since then, more recent data have resulted in the approval of pirfenidone in Europe, and preliminary negative findings regarding the safety of a triple therapy regimen consisting of prednisone, azathioprine and N-acetylcysteine have raised the question of whether it is no longer a treatment option. As clinicians, we must interpret the available guidance and recommendations as we consider each individual patient and as we discuss the available clinical data and the patient’s own preferences in our approach to the management of this disease.
Highlights
The management of patients with Idiopathic Pulmonary Fibrosis (IPF) is largely based on the recommendations of prominent societies, such as the American Thoracic Society (ATS) and the European Respiratory Society (ERS)
The 2011 joint statement of the ATS, ERS, Japanese Respiratory Society (JRS) and Latin American Thoracic Association (ALAT) provided further guidance on the diagnosis of IPF and provided mini-reviews of each of the therapeutic agents that have been used in studies for the treatment of patients with IPF
The treatment recommendations and methodology The most recent guidelines document provided the overall opinion of a voting committee on questions for each therapeutic agent, for example “should patients with IPF be treated with corticosteroid monotherapy?”
Summary
The management of patients with Idiopathic Pulmonary Fibrosis (IPF) is largely based on the recommendations of prominent societies, such as the American Thoracic Society (ATS) and the European Respiratory Society (ERS). The initial recommendations of the ATS/ERS joint committee were made in 2000; the guidance provided stated that at that time there were no existing data to demonstrate the benefit of any treatment for IPF patients. In this paper we discuss the recommendations made by the committee, data that has since become available regarding the use of specific drugs, and suggest the current implications of these for the IPF physician.
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