title: A critical assessment of treatment options for idiopathic pulmonary fibrosis.
abstract: BACKGROUND
  To date, no management approach has proven to be efficacious for the treatment of idiopathic pulmonary fibrosis (IPF). Consequently, therapeutic options remain controversial and confusing for many clinicians. We sought to formally review available evidence on treatment options for IPF and to have a diverse panel of physicians rate the "appropriateness," "inappropriateness," or "uncertainty" of some of the available therapeutic options.
  
  
  METHODS
  The RAND/UCLA Appropriateness Method was used to review and rate multiple clinical scenarios for the treatment of IPF. The panel was composed of nine physicians from geographically diverse areas who received a systematic review on the risks and benefits of commonly used treatments for IPF as background.
  
  
  RESULTS
  A total of 324 clinical scenarios were rated: 25% as appropriate; 39%, uncertain; and 36%, inappropriate. The panel disagreed about 12% of the therapy indications in the final ratings, falling from 26% in the first-round ratings.
  
  
  CONCLUSIONS
  Key themes emerged from the consensus process. Lacking evidence for a definitive therapy, it was considered most appropriate to enroll eligible patients in clinical trials and refer eligible patients for transplant evaluation. For patients without access to clinical trials, the committee was not unanimous regarding treatment recommendations. It was considered inappropriate for patients with a confident diagnosis of IPF to be treated with corticosteroids as the sole agent: corticosteroids should be used in conjunction with azathioprine. With progressive disease despite such combination use, there was agreement for the use of interferon gamma-1b in patients unwilling or unable to participate in available clinical trials.
authors.affiliation: Department of Medicine, New York University School of Medicine, New York, NY, USA. nirav.shah@med.nyu.edu
authors.forename: Nirav R
authors.initials: NR
authors.lastname: Shah
authors.forename: Paul
authors.initials: P
authors.lastname: Noble
authors.forename: Robert M
authors.initials: RM
authors.lastname: Jackson
authors.forename: Talmadge E
authors.initials: TE
authors.lastname: King
authors.forename: Steven D
authors.initials: SD
authors.lastname: Nathan
authors.forename: Maria
authors.initials: M
authors.lastname: Padilla
authors.forename: Ganesh
authors.initials: G
authors.lastname: Raghu
authors.forename: Melissa Bruce
authors.initials: MB
authors.lastname: Rhodes
authors.forename: Marvin
authors.initials: M
authors.lastname: Schwarz
authors.forename: Gregory
authors.initials: G
authors.lastname: Tino
authors.forename: Robert W
authors.initials: RW
authors.lastname: Dubois
mesh_terms: D000368:Aged; D006801:Humans; D008875:Middle Aged; D011658:Pulmonary Fibrosis / Q000175:diagnosis / Q000188:drug therapy* / Q000503:physiopathology