What is the management approach for a patient over 60 with Idiopathic Pulmonary Fibrosis (IPF), possibly with a history of smoking and exposure to lung irritants?

Management of Idiopathic Pulmonary Fibrosis

Initiate antifibrotic therapy with either pirfenidone or nintedanib in all patients with confirmed IPF who have mild-to-moderate disease (FVC ≥50% predicted and DLCO ≥35% predicted), as these medications slow disease progression and reduce FVC decline. 1, 2

Pharmacological Management

Antifibrotic Therapy

• Pirfenidone is approved for mild-to-moderate IPF (FVC <50% predicted and DLCO <35% predicted) at a dose of 2,403 mg/day divided into three doses with food 1, 2
• Pirfenidone reduces the mean decline in FVC by approximately 193 mL compared to placebo at 52 weeks 2
• Nintedanib is an alternative antifibrotic agent that similarly slows disease progression 3, 4
• Both medications must be initiated and supervised by specialist physicians experienced in IPF diagnosis and management 1

Monitoring and Precautions for Pirfenidone

• Perform liver function tests before initiating treatment, then monthly for the first 6 months, and every 3 months thereafter 1
• Smoking must be discontinued prior to and during treatment, as it increases pirfenidone metabolism 1
• Avoid concomitant omeprazole use due to potential pharmacokinetic interactions 1
• Do not administer to patients on fluvoxamine or those with severe hepatic or renal impairment 1
• Common adverse effects include nausea, rash, fatigue, diarrhea, dyspepsia, photosensitivity, and weight loss 1

Preventive Care

Vaccinations

• Administer annual influenza vaccination to all IPF patients 1
• Provide pneumococcal vaccination using the polysaccharide pneumococcal vaccine 1
• These vaccinations are critical as IPF patients face high risk from respiratory infections, similar to other chronic respiratory disease patients 1

Supportive Therapies

Oxygen Therapy

• Prescribe long-term oxygen therapy for patients with severe hypoxemia at rest (severe chronic respiratory failure) 1
• This recommendation is based on survival benefits demonstrated in other hypoxemic lung diseases 1

Pulmonary Rehabilitation

• Initiate a respiratory rehabilitation program in patients with significant exercise limitation and functional impairment 1
• Programs should include exercise training, smoking cessation, psychosocial assistance, and supportive care 1
• Rehabilitation improves walking distance, symptoms, and quality of life 1
• Note that rehabilitation may not be feasible in patients with advanced disease 1

Lung Transplantation Evaluation

• Consider lung transplantation in all patients aged <65 years with severe or worsening disease 1
• Provide information about transplantation early in the disease course 1
• Arrange early assessment at a lung transplantation center 1
• Transplantation improves survival in advanced IPF 1
• Specific criteria include DLCO <39% predicted and FVC decline >10% over 6 months 1
• The age limit of <65 years is relative and depends on local practice and comorbid conditions 1

Management of Acute Exacerbations

Diagnosis

• Diagnose acute exacerbation when there is recent worsening of dyspnea (<30 days) with new lung opacities on imaging, after excluding infection, pulmonary embolism, and left heart failure 1, 5
• HRCT typically shows new ground-glass opacities in addition to pre-existing fibrotic changes 1, 5
• Worsening hypoxemia (≥10 mmHg decrease in PaO2) is common 1, 5

Treatment

• Use high-dose corticosteroids as first-line treatment for acute exacerbations, despite limited controlled trial evidence 1, 5
• Consider intravenous cyclophosphamide as adjunctive immunosuppressive therapy 1, 5
• Avoid invasive mechanical ventilation in patients with established IPF and acute respiratory failure due to high associated mortality 5
• Prefer non-invasive ventilation when respiratory support is needed 5
• Video-assisted surgical lung biopsy is too hazardous during acute exacerbation and should not be performed 1, 5

Important Clinical Considerations

Risk Factors and Demographics

• IPF typically presents in patients over 60 years of age, predominantly male, with a history of cigarette smoking 1
• Consider familial IPF and genetic predisposing factors in younger patients or those with family history of ILD 1
• Gastroesophageal reflux is a common associated risk factor 1

Comorbidity Management

• Evaluate and manage common comorbidities including emphysema, lung cancer, pulmonary hypertension, sleep apnea, and coronary artery disease 1
• Address gastroesophageal reflux disease, which is frequently present 1

Goals of Care

• Discuss goals of care, including decisions about mechanical ventilation, during stable clinic visits before an acute exacerbation occurs 5
• Integrate palliative care early for symptom management and quality of life optimization 6