What is the best treatment approach for a patient with an acute flare of pulmonary fibrosis, possibly with a history of gastroesophageal reflux disease (GERD)?

Treatment of Acute Exacerbation of Pulmonary Fibrosis

The majority of patients with acute exacerbation of pulmonary fibrosis should be treated with high-dose corticosteroids alongside comprehensive supportive care, despite the absence of controlled trial evidence. 1, 2

Primary Pharmacological Management

Corticosteroid Therapy

• Initiate high-dose intravenous corticosteroids as first-line treatment, with doses up to 1 gram per day reported in case series, though specific dosing, route, and duration cannot be definitively recommended based on current evidence. 1, 2
• The recommendation for corticosteroids is based on anecdotal reports of benefit and the extremely high mortality associated with acute exacerbations, not on controlled trial data. 1
• This represents a weak recommendation with very low-quality evidence, meaning the decision should involve shared decision-making with patients regarding goals of care. 1

Adjunctive Immunosuppression

• Consider intravenous cyclophosphamide as adjunctive immunosuppressive therapy in select cases, though evidence is limited. 2
• Cyclosporin A has been used without conclusive results and cannot be routinely recommended. 1

Anticoagulation Considerations

• Do NOT routinely prescribe anticoagulation for acute exacerbation treatment, as warfarin has been associated with increased mortality in IPF patients. 1
• Low-molecular weight heparin may be considered only if thromboembolic disease is suspected and cannot be ruled out. 2

Infection Management

• Administer antibiotics if infection cannot be definitively excluded, as distinguishing infection from acute exacerbation can be challenging. 2

Critical Supportive Care Measures

Respiratory Support

• Non-invasive ventilation (NIV) should be the first-line ventilatory support for patients with acute respiratory failure. 2
• Invasive mechanical ventilation is NOT recommended for the majority of patients with established IPF and acute respiratory failure, given extremely poor outcomes. 1, 2
• Mechanical ventilation may only be appropriate in highly select circumstances: as a bridge to lung transplantation, first manifestation of ILD where diagnosis is uncertain, or acute superimposed infection in otherwise stable disease. 1, 2

Oxygen Supplementation

• Provide supplemental oxygen to maintain adequate oxygenation during the acute phase. 2

Management of Contributing Comorbidities

Gastroesophageal Reflux Disease

• Initiate or optimize antacid therapy with proton pump inhibitors (PPIs) or H2-receptor antagonists, as GERD is highly prevalent (up to 90%) in IPF patients and may contribute to disease progression through microaspiration. 1
• Up to 50% of patients have asymptomatic GERD, making empiric treatment reasonable even without symptoms. 1
• This recommendation is based on observational data showing potential survival benefit (HR 0.47) and smaller FVC decline in patients receiving antacid therapy. 1

Pulmonary Hypertension

• Pulmonary hypertension should generally NOT be treated in most IPF patients during acute exacerbation. 1
• Consider vasomodulatory therapy only in patients with moderate to severe pulmonary hypertension (mean PAP >35 mmHg) documented by right heart catheterization. 1

Post-Exacerbation Recovery and Monitoring

Pulmonary Rehabilitation

• Refer patients for pulmonary rehabilitation after stabilization, as this improves functional status, walk distance, and quality of life. 1, 2
• Benefits may be more pronounced in patients with worse baseline functional status. 1

Disease Monitoring

• Reassess patients every 3-6 months or sooner if clinically indicated with pulmonary function testing (FVC, DLCO) and 6-minute walk testing. 1
• Consider annual high-resolution CT if clinical suspicion of worsening or risk of lung cancer exists. 1
• Recovery may require several months, with ongoing monitoring for progression of fibrosis. 2

Lung Transplantation Considerations

• Evaluate eligible patients for lung transplantation early, particularly those at increased risk of mortality or with severe/worsening disease. 1, 2
• Patients should receive information about lung transplantation early in their disease course, as transplantation improves survival in advanced ILD. 2
• Mechanical ventilation may be considered as a bridge to transplantation in rare, carefully selected circumstances. 1

Critical Pitfalls to Avoid

• Do NOT delay recognition and treatment of acute exacerbation, as early intervention is crucial for outcomes. 2
• Avoid overuse of mechanical ventilation in patients with advanced disease, where it may prolong suffering without improving outcomes. 2
• Do NOT use corticosteroid monotherapy for chronic IPF management outside of acute exacerbations, as it causes substantial long-term morbidity without proven survival benefit. 1
• Ensure infection is adequately ruled out before attributing respiratory decline solely to acute exacerbation, as this distinction significantly impacts treatment decisions. 2