विवरण
Interstitial lung disease (ILD) is a broad term used to describe a group of chronic lung conditions. An accurate diagnosis of ILD requires a thorough medical history, physical examination, and imaging tests. High-resolution computed tomography (HRCT) scans provide detailed images of the lungs and help to identify specific patterns of lung disease. The treatment of ILD depends on the underlying cause and can include medications, oxygen therapy, and lifestyle changes. In some cases, surgical interventions such as lung transplantation may be required.
सारांश
- Interstitial lung diseases (ILDs) are a diverse group of disorders characterized by generalized involvement of the lung interstitium. It is important to first classify whether an ILD is idiopathic pulmonary fibrosis (IPF) or non-IPF due to the significantly poorer prognosis of IPF. Early and accurate diagnosis is critical for timely initiation of appropriate therapy and improved patient outcomes.
- A thorough patient history, including the chronicity of symptoms like breathlessness and cough, is crucial for initial assessment. Physical examination findings such as fine "velcro-like" crackles and clubbing of the fingers are highly suggestive of ILD. Pulmonary function tests (PFTs) and exertional desaturation can further support the diagnosis.
- Radiology plays a central role in ILD diagnosis, with chest X-rays potentially showing interstitial shadows, and high-resolution computed tomography (HRCT) scans providing detailed information. Patterns to look for include reticulation, nodulation, high attenuation (consolidation), and low attenuation (cysts). Identifying the distribution of these patterns (central, perilymphatic, upper zone, lower zone) aids in narrowing the differential diagnosis.
- Secondary pulmonary lobules are key to understanding CT patterns, with the periphery containing veins and lymphatics and the center containing airways and arteries. Septal thickening, often seen in ILD, highlights these interlobular septa. Honeycombing (multiple layers of subpleural cysts) and traction bronchiectasis (dilated airways due to fibrosis) are hallmark features in certain ILDs.
- Idiopathic interstitial pneumonias (IIPs) are classified based on presentation and patterns into chronic fibrosing, smoking-related, and acute/subacute categories. IPF is the most important subtype due to its aggressive nature and poor prognosis. The histological and radiological hallmark of IPF is the usual interstitial pneumonia (UIP) pattern.
- The UIP pattern on HRCT includes subpleural and basal predominant distribution, often heterogeneous, with honeycombing with or without peripheral traction bronchiectasis. Probable UIP lacks honeycombing but retains other features. Indeterminate UIP lacks definitive features of UIP, necessitating further investigation. Ruling out alternative diagnoses such as asbestosis, sarcoidosis, and drug-induced ILD is crucial in diagnosing IPF.
- Management of IPF focuses on slowing disease progression and maintaining quality of life. Non-pharmacological approaches include patient education, referral to specialist centers, oxygen therapy, pulmonary rehabilitation, and management of comorbidities. The antifibrotic drugs pirfenidone and nintedanib are approved for slowing the rate of decline in lung function.
- Pirfenidone and nintedanib are antifibrotic drugs that limit collagen synthesis and slow FVC decline but cannot stop disease progression. Both medications can have side effects, including nausea, diarrhea, photosensitivity and gastric upsets, often requiring careful dose titration. Regular monitoring with PFTs, 6-minute walk tests, and CT scans helps assess treatment response. Lung transplantation is the only definitive treatment for IPF.
- Other IIPs include nonspecific interstitial pneumonia (NSIP), respiratory bronchiolitis-associated ILD (RB-ILD), and desquamative interstitial pneumonia (DIP), often linked to smoking. Organizing pneumonia typically responds well to corticosteroids. Lymphoid interstitial pneumonia (LIP) is associated with HIV and lymphoma. Drug-induced ILD must always be considered in the differential diagnosis.
नमूना प्रमाण पत्र
वक्ताओं के बारे में
Dr. Rajneesh kumar Srivastava
Consultant - Respiratory and Sleep Medicine Lucknow
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