From the Guidelines
The diagnostic approach for Interstitial Lung Disease (ILD) should begin with a multidisciplinary assessment of clinical, laboratory, imaging, functional, and sometimes histologic features, with a thorough history and physical examination, including pulmonary function tests (PFTs) and high-resolution computed tomography (HRCT) as essential tools for early detection and diagnosis. The initial evaluation of all patients with suspected ILD should include a complete history and physical examination, focusing on occupational exposures, medication use, and systemic symptoms, as well as a search for clinical features suggestive of connective tissue disease (CTD) 1. The presence of ‘velcro’ crackles in lung auscultation suggests the presence of lung fibrosis and should be addressed and considered if present. In the case of interstitial pneumonia, an autoimmune panel should be performed to investigate a hypothetical underlying CTD. High-resolution computed tomography (HRCT) is the primary imaging tool used to detect ILD and outline its extension and pattern, with a volumetric HRCT scan acquired on full inspiration, complemented at baseline with an additional acquisition in ventral decubitus and a non-contiguous acquisition on expiration 1. Pulmonary function tests (PFTs) are also a reliable tool for early ILD detection, providing the most accurate measurement of disease severity, and serial PFTs should be performed as part of a close-follow-up strategy 1. A multidisciplinary evaluation of disease severity, associated symptoms, pulmonary function impairment, and disease extent on HRCT is crucial for accurate diagnosis and management of ILD, as supported by recent guidance on the early recognition and follow-up of patients with connective tissue disease-related interstitial lung disease 1. Key considerations in the diagnostic workup include:
- A thorough history and physical examination
- Pulmonary function tests (PFTs) with spirometry, lung volumes, and diffusion capacity
- High-resolution computed tomography (HRCT) of the chest
- Laboratory studies, including complete blood count, comprehensive metabolic panel, erythrocyte sedimentation rate, C-reactive protein, and serologic testing for autoimmune diseases
- Bronchoscopy with bronchoalveolar lavage and surgical lung biopsy via video-assisted thoracoscopic surgery (VATS) as needed for definitive diagnosis.
From the FDA Drug Label
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From the Research
Diagnostic Approach for Interstitial Lung Disease (ILD)
The diagnostic approach for ILD involves a combination of clinical evaluation, laboratory testing, and imaging studies. The following steps are involved in the diagnostic process:
- A thorough medical history and detailed physical examination to identify symptoms and signs of ILD, such as non-productive cough and exertional dyspnea 2, 3, 4
- Laboratory testing, including blood gas analysis and serological tests to identify underlying conditions such as connective tissue disease 2, 3, 4
- Chest radiography and high-resolution computed tomography (HRCT) to evaluate the extent and pattern of lung involvement 2, 3, 4, 5
- Pulmonary function testing, including spirometry and diffusion capacity for carbon monoxide, to assess lung function 2, 3, 6
- A multidisciplinary discussion (MDD) between pulmonologists, radiologists, and pathologists to interpret HRCT scans and establish a diagnosis 5
Role of HRCT in ILD Diagnosis
HRCT plays a crucial role in the diagnosis of ILD, as it can help identify specific patterns of lung involvement and distinguish between different types of ILD 2, 3, 4, 5. The interpretation of HRCT scans requires close communication between pulmonologists and radiologists, and a common lexicon to describe HRCT features can help improve the effectiveness of this communication 5.
Monitoring and Follow-up
Once a diagnosis of ILD is established, regular monitoring and follow-up are essential to assess disease progression and response to treatment 2, 6. This may involve repeated clinical evaluations, laboratory testing, and imaging studies, as well as symptom tracking and quality of life assessments 6.