What is the next step from a pulmonary standpoint for a 60-year-old male (M) with possible leukemia and a computed tomography (CT) scan suggesting pulmonary fibrosis, likely interstitial pneumonia or idiopathic interstitial fibrosis?

From the Guidelines

The next step from a pulmonary standpoint for this 60-year-old male with possible leukemia and CT findings suggestive of pulmonary fibrosis/interstitial pneumonia would be a comprehensive pulmonary evaluation including pulmonary function tests (PFTs) with diffusion capacity and a high-resolution CT scan (HRCT) if not already performed, as recommended by the most recent guidelines 1. This approach is crucial because the patient's CT scan suggests interstitial pneumonia or idiopathic interstitial fibrosis, which requires further evaluation to determine the specific type of interstitial lung disease.

• A multidisciplinary discussion involving pulmonology, radiology, and potentially rheumatology should be conducted to determine if a surgical lung biopsy is necessary for definitive diagnosis, considering the patient's age and the presence of possible leukemia.
• The patient should undergo a 6-minute walk test to assess exercise capacity and oxygen requirements, as this will provide valuable information on the patient's functional status and guide treatment decisions.
• Laboratory testing should include autoimmune markers (ANA, RF, anti-CCP), given the possibility of connective tissue disease-associated interstitial lung disease, as suggested by the guidelines 1.
• While awaiting definitive diagnosis, supplemental oxygen should be provided if hypoxemia is present, to alleviate symptoms and improve quality of life.
• The evaluation needs to be expedited as the patient's possible leukemia may impact treatment options, and early referral to an interstitial lung disease specialist is recommended, as management may include antifibrotic medications like pirfenidone or nintedanib if idiopathic pulmonary fibrosis is confirmed, or immunosuppressive therapy if inflammatory pathology is identified, as updated in the recent guidelines 1.
• The recent guidelines also emphasize the importance of a multidisciplinary discussion in determining the diagnosis and treatment of interstitial lung diseases, including idiopathic pulmonary fibrosis and progressive pulmonary fibrosis 1.
• Accurate diagnosis of the specific type of interstitial lung disease is crucial for appropriate treatment selection and prognosis determination, and the comprehensive approach outlined above is essential to achieve this goal, as supported by the evidence 1.

From the FDA Drug Label

The primary endpoint was the change in percent predicted forced vital capacity (%FVC) from baseline to study end, measured at 52 weeks in Study 1, and at 72 weeks in Studies 2 and 3 Eligible patients were to have %FVC greater than or equal to 50% at baseline and a percent predicted diffusing capacity of the lungs for carbon monoxide (%DLCO) greater than or equal to 30% (Study 1) or 35% (Studies 2 and 3) at baseline.

The next step from a pulmonary standpoint for a 60-year-old male with possible leukemia and a CT scan indicating likely interstitial pneumonia or idiopathic interstitial fibrosis would be to conduct pulmonary function tests (PFTs), including forced vital capacity (FVC) and diffusing capacity of the lungs for carbon monoxide (DLCO), to determine the extent of lung involvement and to assess the patient's eligibility for potential treatment with pirfenidone 2.

• Pulmonary function tests will help to determine the patient's %FVC and %DLCO values.
• These values will help guide the treatment approach and determine if the patient meets the criteria for treatment with pirfenidone.

From the Research

Next Steps from a Pulmonary Standpoint

Given the CT scan results indicating possible pulmonary fibrosis, likely interstitial pneumonia, or idiopathic interstitial fibrosis in a 60-year-old male with suspected leukemia, the next steps would involve a comprehensive evaluation to confirm the diagnosis and assess the extent of the disease.

• Multidisciplinary Diagnosis: As emphasized by 3, a multidisciplinary approach integrating clinical, radiological, and pathologic findings is crucial for the accurate diagnosis of idiopathic pulmonary fibrosis (IPF) and differentiation from interstitial lung abnormality (ILA) or other interstitial lung diseases.
• High-Resolution Computed Tomography (HRCT): The use of HRCT is pivotal, as it allows for the detailed assessment of interstitial compartments and can provide patterns suggestive of underlying pathology, such as usual interstitial pneumonia (UIP) 4.
• Clinical Evaluation: Proper clinical evaluation is necessary to differentiate between ILA and clinically significant interstitial lung disease (ILD), considering factors such as symptoms, exposure history, and the presence of other diseases like leukemia 3.
• Pattern Classification: Understanding the CT scan patterns, such as probable UIP or indeterminate for UIP, is important for prognosis, as certain patterns may be associated with a worse prognosis 5.
• Pathologic Patterns: Recognizing the three major pathologic patterns of pulmonary fibrosis (UIP, nonspecific interstitial pneumonia (NSIP), and airway-centered fibrosis (ACF)) and their corresponding radiographic patterns can aid in diagnosis 6.
• Diagnostic Certainty: The integrated clinico-radiological assessment can influence diagnostic certainty, and alternative diagnoses such as chronic hypersensitivity pneumonitis (CHP), interstitial pneumonia associated with collagen tissue diseases (CTD-IP), and idiopathic NSIP should be considered 7.
• Multidisciplinary Team Discussion: A discussion among a multidisciplinary team of experts, including radiologists and pulmonologists, is essential to reach a consensus on the diagnosis and to plan appropriate management strategies.