ATS GUIDELINES Bundle

INSIDE ▶ Introduction ▶ Recommendations ▶ Tables ▶ Figures Idiopathic Pulmonary Fibrosis and Progressive Pulmonary Fibrosis

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11 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

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