title: Pharmacological management of IPF.
abstract: Idiopathic pulmonary fibrosis (IPF) is a deadly disease with a median survival of approximately three years in historical cohorts. Despite increased knowledge of disease pathophysiology and selection of more targeted therapy, main clinical trials yielded negative results. However, two agents, pirfenidone and nintedanib, were recently shown to be effective in IPF and received marketing authorization worldwide. Both drugs significantly reduce functional decline and disease progression with an acceptable safety profile. Yet, none of these drugs actually improves or even stabilizes the disease or the symptoms perceived by the patient. Several other treatments and combinations are currently tested, and many more are ready for clinical trials. Their completion is critical for achieving the ultimate goal of curing patients with IPF.
authors.affiliation: APHP, Bichat Hospital, Department of Pneumology A, Centre de competence des maladies pulmonaires rares, DHU Fire, Paris, France.|INSERM, Paris, France.|Paris Diderot University, Paris, France.
authors.forename: Raphael
authors.initials: R
authors.lastname: Borie
authors.affiliation: APHP, Bichat Hospital, Department of Pneumology A, Centre de competence des maladies pulmonaires rares, DHU Fire, Paris, France.|INSERM, Paris, France.|Paris Diderot University, Paris, France.
authors.forename: Aurelien
authors.initials: A
authors.lastname: Justet
authors.affiliation: APHP, Bichat Hospital, Department of Pneumology A, Centre de competence des maladies pulmonaires rares, DHU Fire, Paris, France.|INSERM, Paris, France.|Paris Diderot University, Paris, France.
authors.forename: Guillaume
authors.initials: G
authors.lastname: Beltramo
authors.affiliation: 2nd Respiratory Medicine Department, 'Attikon' University Hospital, Athens Medical School, National and Kapodistrian University of Athens, Athens, Greece.
authors.forename: Effrosyni D
authors.initials: ED
authors.lastname: Manali
authors.affiliation: APHP, Bichat Hospital, Department of Pneumology A, Centre de competence des maladies pulmonaires rares, DHU Fire, Paris, France.|INSERM, Paris, France.|Paris Diderot University, Paris, France.
authors.forename: Pauline
authors.initials: P
authors.lastname: Pradère
authors.affiliation: Clinica di Malattie dell'Apparato Respiratorio, Università degli Studi di Padova, Padova, Italy.
authors.forename: Paolo
authors.initials: P
authors.lastname: Spagnolo
authors.affiliation: APHP, Bichat Hospital, Department of Pneumology A, Centre de competence des maladies pulmonaires rares, DHU Fire, Paris, France.|INSERM, Paris, France.|Paris Diderot University, Paris, France.
authors.forename: Bruno
authors.initials: B
authors.lastname: Crestani
mesh_terms: D018450:Disease Progression; D006801:Humans; D054990:Idiopathic Pulmonary Fibrosis / Q000175:diagnosis / Q000188:drug therapy* / Q000503:physiopathology; D056747:Immunomodulation / Q000187:drug effects*; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D007211:Indoles / Q000627:therapeutic use*; D008297:Male; D008875:Middle Aged; D017410:Practice Guidelines as Topic; D011728:Pyridones / Q000627:therapeutic use*; D016032:Randomized Controlled Trials as Topic; D016896:Treatment Outcome
chemical_list: D007166:Immunosuppressive Agents; D007211:Indoles; D011728:Pyridones; C093844:pirfenidone; C530716:nintedanib
keywords: cough; exacerbation; nintedanib; pirfenidone; pulmonary hypertension