From the Guidelines
The medical diagnosis for an X-ray result indicating Interstitial Lung Disease (ILD) should be based on a multidisciplinary assessment of clinical, laboratory, imaging, functional, and sometimes histologic features, as recommended by the most recent guidelines 1. When ILD is suspected on X-ray, a high-resolution CT scan (HRCT) should be ordered immediately for confirmation and better characterization of the disease pattern. The clinical evaluation must include a search for clinical features suggestive of connective tissue disease (CTD), and the presence of ‘velcro’ crackles in lung auscultation suggests the presence of lung fibrosis and should be addressed and considered if present.
Key Considerations
- A complete history and physical examination are essential in the initial evaluation of patients with suspected ILD.
- The clinical evaluation should include a search for clinical features suggestive of CTD.
- High-resolution computed tomography (HRCT) is the primary imaging tool used to detect ILD and outline its extension and pattern.
- Pulmonary function tests (PFTs) are a reliable tool for early ILD detection and provide the most accurate measurement of disease severity.
- Patients diagnosed with ILD should undergo a multidisciplinary evaluation of disease severity, associated symptoms, pulmonary function impairment, and disease extent on HRCT.
Treatment Approach
Treatment depends on the specific type of ILD, but often includes:
- Corticosteroids like prednisone for inflammatory forms
- Antifibrotics such as pirfenidone or nintedanib for idiopathic pulmonary fibrosis
- Immunosuppressants like mycophenolate mofetil or azathioprine for connective tissue disease-related ILD Supportive care is essential, including supplemental oxygen, pulmonary rehabilitation, smoking cessation, and vaccination against pneumococcal pneumonia and influenza, as recommended by recent guidelines 1.
Underlying Pathophysiology
The underlying pathophysiology involves inflammation and/or fibrosis of the lung interstitium, which impairs gas exchange and leads to restrictive lung function, as described in previous studies 1. Early diagnosis and treatment are crucial as some forms of ILD can progress rapidly, causing irreversible lung damage.
From the FDA Drug Label
Studies 1,2 and 3 enrolled adult patients who had a clinical and radiographic diagnosis of IPF (with or without accompanying surgical lung biopsy), without evidence or suspicion of an alternative diagnosis for interstitial lung disease
The medical diagnosis for an X-ray result indicating Interstitial Lung Disease (ILD) is Idiopathic Pulmonary Fibrosis (IPF), as the studies enrolled patients with a clinical and radiographic diagnosis of IPF. 2
From the Research
Medical Diagnosis for Interstitial Lung Disease (ILD)
The medical diagnosis for an X-ray result indicating Interstitial Lung Disease (ILD) involves a multidisciplinary approach, including:
- Key history and examination features
- Blood panel
- Pulmonary function tests
- High-resolution computed tomography (HRCT) imaging
- Bronchoalveolar lavage and lung biopsy results, when required 3 A diagnosis of ILD can be reached through a combination of these factors, and a holistic approach to patient care is essential due to the high symptom burden and palliative care needs of patients with ILD.
Classification and Management of ILD
ILD can be classified into different categories, including:
- Non-subpleural, subpleural non-fibrotic, and subpleural fibrotic patterns based on HRCT findings 4
- Patients can be classified as having ILA, at high risk for developing ILD, probable ILD, or ILD based on symptoms, physiologic assessment, and risk factors 4 Management of ILD is disease-specific, and a multidisciplinary discussion is recommended to evaluate features that indicate an increased risk of progression 4
Diagnostic Algorithms for ILD
Several diagnostic algorithms have been proposed for ILD, including:
- The Interstitial Lung Disease Reporting And Data System (ILD-RADS) framework 5
- An algorithmic approach to the identification and management of patients with interstitial lung abnormalities (ILAs) 4 These algorithms aim to aid clinicians in interpreting radiologic patterns and reaching a diagnosis of ILD.
Prevalence and Risk Factors for ILD
ILD is a heterogeneous group of lung disorders with characteristic clinical and imaging features, and patients with ILD are at an increased risk of developing non-small cell lung cancer (NSCLC) 6 The prevalence of ILD in patients with connective tissue diseases is high, with HRCT abnormalities suggestive of ILD observed in 85.1% of patients 7