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Treatment considerations should include both pharmacological (nintedanib and pirfenidone)
and nonpharmacological (oxygen supplementation, pulmonary rehabilitation) therapies. Patients
should be evaluated and treated for existing comorbidities, including pulmonary hypertension,
gastroesophageal reflux, obstructive sleep apnea, and lung cancer. Patients may benefit from
involvement of palliative care to help with symptom management (cough, dyspnea, anxiety).
Patient values and preferences should be explored. Patients at increased risk of mortality should
be referred for lung transplantation at diagnosis. Patients should be evaluated every 3–6 months
or more oen for disease progression. Acute exacerbations may be treated with corticosteroids.
Mechanical ventilation is not recommended for the majority of patients with respiratory failure.
Monitor For Disease Progression
Consider pulmonary function testing
and the 6-minute walk test every 4–6
months or sooner if clinical indicated
Consider annual HRCT if there is
clinical suspicion of worsening or
risk of lung cancer
Consider an HRCT if there is concern
for an acute exacerbation
Consider a CT pulmonary angiogram
if there is a clinical concern for
pulmonary embolism
Acute Exacerbation
Corticosteroids
Respiratory Failure Due to
Progression of IPF
Evaluate and
list for lung
transplantation
Palliative care
