title: Challenges in IPF diagnosis, current management and future perspectives.
abstract: Recent developments have clarified our understanding of IPF and improved outcomes with two viable new therapeutic options, pirfenidone and nintedanib. In spite of these advances, questions and challenges concerning IPF still remain. Here we will focus on some of these unresolved areas: the diagnosis of IPF is hindered by limitations in current practice guidelines, surgical lung biopsy is contraindicated in many patients, the accuracy of prognostic evaluation needs to be increased and tolerability factors can jeopardise adherence to treatment. We will also identify new developments shaping the future of IPF management such as cryobiopsy, increased understanding of genetic factors and new treatment paradigms, which may help to fulfil currently unmet needs.
authors.affiliation: Royal Brompton Hospital, London, UK. redazione@mattioli1885.com.
authors.forename: Athol U
authors.initials: AU
authors.lastname: Wells
authors.forename: Ulrich
authors.initials: U
authors.lastname: Costabel
authors.forename: Venerino
authors.initials: V
authors.lastname: Poletti
authors.forename: Bruno
authors.initials: B
authors.lastname: Crestani
authors.forename: Jim
authors.initials: J
authors.lastname: Egan
authors.forename: George
authors.initials: G
authors.lastname: Margaritopoulos
authors.forename: Katerina
authors.initials: K
authors.lastname: Antoniou
mesh_terms: D000368:Aged; D001707:Biopsy, Needle / Q000009:adverse effects; D019468:Disease Management; D018450:Disease Progression; D005260:Female; D006801:Humans; D054990:Idiopathic Pulmonary Fibrosis / Q000175:diagnosis* / Q000401:mortality / Q000628:therapy*; D007150:Immunohistochemistry; D008297:Male; D008875:Middle Aged; D020380:Needs Assessment; D017410:Practice Guidelines as Topic; D011379:Prognosis; D011728:Pyridones / Q000627:therapeutic use*; D012129:Respiratory Function Tests; D018570:Risk Assessment; D012720:Severity of Illness Index; D016019:Survival Analysis; D014057:Tomography, X-Ray Computed / Q000379:methods
chemical_list: D011728:Pyridones; C093844:pirfenidone