What is the initial management in the Emergency Department (ED) for a patient with newly diagnosed pulmonary fibrosis presenting with shortness of breath?

Initial ED Management of Newly Diagnosed Pulmonary Fibrosis

For a patient presenting to the ED with newly diagnosed pulmonary fibrosis and worsening shortness of breath, the priority is oxygen supplementation to maintain SpO2 94-98%, ruling out acute reversible causes (infection, pulmonary embolism, heart failure), and avoiding empiric steroids or antibiotics unless specific indications exist. 1, 2

Immediate Assessment and Oxygen Therapy

Provide supplemental oxygen immediately if the patient is hypoxemic:

• Target SpO2 of 94-98% in patients without risk factors for hypercapnia 1
• Start with nasal cannula at 2-6 L/min or simple face mask at 5-10 L/min 1
• If SpO2 is below 85%, use a reservoir mask at 15 L/min 1
• Measure respiratory rate and heart rate carefully, as tachypnea and tachycardia are more common than visible cyanosis in hypoxemic patients 1

Rule Out Acute Exacerbation and Alternative Diagnoses

Before attributing symptoms solely to chronic pulmonary fibrosis, you must exclude:

Acute Exacerbation of IPF

• Defined as acute worsening of dyspnea within <30 days with new ground-glass opacities on imaging 1, 2
• Requires exclusion of infection, pulmonary embolism, left heart failure, and cardiac arrhythmia 1, 2
• Worsening hypoxemia (≥10 mmHg decrease in PaO2) is common 1

Other Reversible Causes

• Infection: Check for fever, leukocytosis, purulent sputum; obtain blood cultures and consider sputum culture 1
• Pulmonary embolism: Assess Wells score, consider D-dimer and CT pulmonary angiography if clinically indicated 1
• Heart failure: Evaluate for volume overload, obtain BNP/NT-proBNP, consider echocardiography 1

Steroid Use: Only for Confirmed Acute Exacerbation

Do NOT give empiric steroids for stable chronic pulmonary fibrosis in the ED. 1

Steroids are indicated ONLY if acute exacerbation is confirmed:

• High-dose corticosteroids are commonly used for acute exacerbations despite limited controlled trial evidence 1, 2
• This decision should ideally be made in consultation with pulmonology 2
• The evidence for steroids in acute exacerbation is weak, but they remain standard practice 1

Important caveat: Chronic steroid use in stable IPF is NOT recommended and may be harmful 1

Antibiotic Use: Only When Infection Cannot Be Excluded

Do NOT give empiric antibiotics for pulmonary fibrosis alone. 1

Antibiotics are indicated only when:

• Infection has not been definitively ruled out as the cause of acute deterioration 1
• Use broad-spectrum coverage if infection is suspected 1
• Clinical signs suggesting infection include fever, elevated white blood cell count, new infiltrates beyond baseline fibrosis, or purulent secretions 1

Avoid Mechanical Ventilation Unless Specific Criteria Met

Invasive mechanical ventilation is NOT recommended for most patients with IPF and acute respiratory failure due to extremely high mortality (>90%). 1, 2

Ventilation may be considered only in highly selected cases:

• As a bridge to emergency lung transplantation in eligible candidates 1, 2
• If the exacerbation is the first manifestation of IPF (not yet established diagnosis) 1, 2
• If there is an acute reversible cause (infection, pulmonary embolism) 1, 2
• Non-invasive ventilation may be preferred over invasive ventilation when respiratory support is deemed necessary 1, 2

ED Disposition and Follow-up

Arrange urgent pulmonology consultation:

• Patients with newly diagnosed pulmonary fibrosis require specialized evaluation for definitive diagnosis and treatment planning 3, 4
• Antifibrotic therapy (pirfenidone or nintedanib) should be initiated by pulmonology to slow disease progression 3, 4, 5
• Early referral for lung transplant evaluation should be considered in eligible patients (<65 years) 1

Initiate supportive measures:

• Prescribe supplemental oxygen for home use if SpO2 <88% at rest or with exertion 1
• Consider proton pump inhibitor for gastroesophageal reflux, which is highly prevalent in IPF 1
• Recommend influenza and pneumococcal vaccination 1

Key Pitfalls to Avoid

• Do not assume all dyspnea is from chronic fibrosis—always rule out acute reversible causes first 1, 2
• Do not give empiric steroids without confirming acute exacerbation, as chronic steroid use in stable IPF lacks benefit and has significant risks 1
• Do not intubate reflexively—discuss goals of care and prognosis, as mechanical ventilation has dismal outcomes in IPF 1, 2
• Do not delay pulmonology referral—early antifibrotic therapy improves outcomes 3, 4