What is the appropriate management for a patient with abnormal Pulmonary Function Test (PFT) results indicating lung dysfunction?

From the Guidelines

Management of a patient with abnormal Pulmonary Function Test (PFT) results indicating lung dysfunction should prioritize a comprehensive clinical evaluation, including history, physical examination, and review of PFT results to determine the underlying cause, and treatment should be tailored to the specific pattern of dysfunction identified, with consideration of pharmacological and nonpharmacological therapies, as well as palliative care and patient values and preferences 1.

Clinical Evaluation

A thorough clinical evaluation is essential to determine the underlying cause of the abnormal PFT results. This includes a comprehensive history, physical examination, and review of PFT results. The evaluation should also consider existing comorbidities, such as pulmonary hypertension, gastroesophageal reflux, obstructive sleep apnea, and lung cancer.

Treatment Approach

The treatment approach should be tailored to the specific pattern of dysfunction identified. For obstructive patterns, bronchodilator therapy is typically first-line, while for restrictive patterns, treatment addresses the underlying cause, which might include immunosuppressants for interstitial lung disease.

• Pharmacological therapies, such as nintedanib and pirfenidone, may be considered for patients with idiopathic pulmonary fibrosis (IPF) 1.
• Nonpharmacological therapies, such as oxygen supplementation and pulmonary rehabilitation, should also be considered.
• Palliative care should be involved to help with symptom management, including cough, dyspnea, and anxiety.

Monitoring and Follow-up

Regular follow-up with serial PFTs every 3-12 months helps monitor disease progression and treatment response. High-resolution computed tomography (HRCT) chest may be useful for monitoring interstitial lung disease (ILD) progression, especially when there is respiratory symptom progression with stable PFTs, a significant reduction in PFTs, screening for lung cancer, or differentiating between tight skin or respiratory muscle weakness versus progressive ILD as the cause of a restrictive PFT pattern 1.

• HRCT chest requires an experienced radiologist or other qualified professional to compare to previous HRCT scans to assess for progression.
• The frequency of HRCT chest monitoring should be determined by patient symptoms and mindful consideration of radiation exposure.

Patient-Centered Care

Patient values and preferences should be explored, and treatment decisions should be made in accordance with these values. Patients at increased risk of mortality should be referred for lung transplantation at diagnosis.

• Supplemental oxygen should be prescribed when resting or exertional hypoxemia is present (SpO2 <88%).
• Pulmonary rehabilitation is beneficial for most patients with chronic lung dysfunction, typically involving 6-12 weeks of supervised exercise and education.

From the FDA Drug Label

In controlled 12-week studies in patients with bronchospasm associated with chronic obstructive pulmonary disease (chronic bronchitis and emphysema) significant improvements in pulmonary function (FEV1 increases of 15% or more) occurred within 15 to 30 minutes, reached a peak in 1 to 2 hours, and persisted for periods of 4 to 5 hours in the majority of patients, with about 25% to 38% of the patients demonstrating increases of 15% or more for at least 7 to 8 hours. Controlled clinical trials, most patients exhibited an onset of improvement in pulmonary function within 5 minutes as determined by FEV1. The primary efficacy analysis for the change in %FVC from baseline to Week 52 demonstrated a statistically significant treatment effect of pirfenidone 2,403 mg/day (n=278) compared with placebo (n=277) using a rank ANCOVA with the lowest rank imputation for missing data due to death

The appropriate management for a patient with abnormal Pulmonary Function Test (PFT) results indicating lung dysfunction may include:

• Bronchodilator therapy: such as ipratropium bromide or albuterol, which can help improve pulmonary function by relaxing bronchial smooth muscle and increasing airflow to the lungs 2, 3.
• Anti-fibrotic therapy: such as pirfenidone, which can help reduce the decline in lung function in patients with idiopathic pulmonary fibrosis (IPF) 4.
• Oxygen therapy: to help increase oxygen levels in the blood and improve symptoms such as shortness of breath.
• Pulmonary rehabilitation: a comprehensive program that includes exercise, education, and support to help improve lung function and overall health. Key considerations in managing patients with abnormal PFT results include:
• Monitoring lung function: regularly assessing PFT results to track changes in lung function and adjust treatment as needed.
• Optimizing medication: ensuring that patients are taking their medications as prescribed and adjusting doses or adding new medications as needed to achieve optimal control of symptoms.
• Addressing underlying conditions: managing underlying conditions that may be contributing to lung dysfunction, such as chronic obstructive pulmonary disease (COPD) or IPF.

From the Research

Management of Abnormal PFT Results

Abnormal Pulmonary Function Test (PFT) results indicating lung dysfunction require appropriate management to prevent further deterioration and improve patient outcomes. The management approach depends on the underlying cause of the lung dysfunction.

Treatment Options

• For idiopathic pulmonary fibrosis (IPF), treatment options include antifibrotic agents such as pirfenidone and nintedanib, which can slow down disease progression 5, 6.
• Inhaled corticosteroids and bronchodilators may be effective in treating lymphocytic interstitial pneumonia (LIP) associated with primary Sjögren's syndrome 7.
• For patients with chronic obstructive pulmonary disease (COPD), inhaled corticosteroids such as fluticasone propionate may improve lung function and reduce airway resistance 8.

Non-Pharmacologic Interventions

• Long-term oxygen therapy, pulmonary rehabilitation, and lung transplantation may be considered as additional treatment modalities for patients with IPF 5.
• Pulmonary function testing (PFT) can help identify underlying pulmonary disease in patients presenting with dysphonia, and may lead to new pulmonary diagnoses or changes in medications 9.

Key Considerations

• The choice of treatment depends on the underlying cause of lung dysfunction and the patient's individual needs.
• A comprehensive diagnostic evaluation, including PFT and other tests, is essential to determine the best course of treatment.
• Patients with abnormal PFT results should be referred to a pulmonologist for further evaluation and management 9.