Beyond the Guidelines Question [Survey]

Best Practices


A. Assume that using best practices for diagnosing and treating idiopathic pulmonary fibrosis (IPF) means all of the following:

  • Accurately diagnosing IPF through the exclusion of other known causes of interstitial lung disease (eg, environmental exposures, autoimmune disease, drug toxicity) with physical exam, history, and lab evaluations; and the use of high-resolution computed tomography (HRCT), with or without lung biopsy
  • Using a multidisciplinary team approach including pulmonologists, radiologists, and pathologists experienced in the diagnosis of interstitial lung disease (ILD)
  • Making timely referral to specialty ILD care
  • Appropriately using nonpharmacological therapies such as oxygen and pulmonary rehabilitation to improve quality of life
  • Engaging in shared decision-making with the patient to select appropriate pharmacological therapy (nintedanib or pirfenidone) if indicated, and avoiding therapies known to have no benefit and/or cause harm (N-acetylcysteine, azathioprine, and prednisone)
  • Referring appropriate candidates for consultation about lung transplantation upon diagnosis
  • Being proactive in the recognition, referral, and management of common comorbidities such as gastroesophageal reflux disease (GERD), sleep apnea, and depression
  • Communicating early and effectively with patients about disease state, prognosis, goals of care, palliative care, and end of life issues
  • Providing supportive patient resources
  • Monitoring for disease progression and assessing for reversible and treatable causes of shortness of breath, hypoxia, and interstitial infiltrate
Not importantA little importantModerately importantImportantExtremely important
Improves patients' quality of life *
Can limit a clinician’s autonomy *
Offers a more of consistent approach to patient care *
Is challenging if the practice staff does not have the band width to implement them *
Encourages shared decision-making *
Requires extra effort *
Ensures that patients have access to support groups, emerging therapies, and clinical trials *
Can be prohibitive with burdensome costs to patients *



The next section lists situations in which some clinicians might not use best practices for diagnosing and treating IPF. Please tell us how confident you are that you would use best practices in these situations.

Not at all confidentSomewhat confidentModerately confidentConfidentExtremely confident
If it is unclear who has primary responsibility for managing a patient *
If you lack the resources or access to multidisciplinary care *
If there is not enough time to educate patients about disease progression and treatment options *
If patients report side effects of pharmacologic therapy *
If you’re uncertain about when to have a meaningful conversation with patients about their values and priorities *