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Interstitial Lung Diseases

Cardio-thoracic SurgeryRespiratory Medicine

Interstitial Lung Disease (ILD) is a group of disorders that affect the interstitium of the lungs (the tissue surrounding the alveolar walls and their surrounding structures). The primary characteristics of ILD are inflammation and fibrosis of the lung tissue, resulting in impaired gas exchange. Interstitial Lung Disease can be idiopathic, known causes include autoimmune diseases, certain medications, prolonged exposure to harmful substances, radiation, and lung infections. Symptoms include breathlessness on exertion and a persistent dry cough. Certain conditions linked to interstitial lung disease may cause additional symptoms in other body parts, such as skin or joint issues in autoimmune disorders.

Symptoms

Early interstitial lung disease (ILD) symptoms may be subtle, presenting only as mild shortness of breath or occasional dry cough, and are often overlooked. Many patients seek medical attention once the disease has progressed to moderate or advanced stages with more pronounced symptoms. Key symptoms include:

Shortness of Breath (Dyspnoea)
Early Stage: Typically occurs during exercise or physical activity.
Late Stage: May be present even at rest. Shortness of breath is the most characteristic symptom and worsens progressively.
Chronic Dry Cough
A persistent, non-infectious dry cough lasting weeks to months, often resistant to treatment.
Usually unaccompanied by mucus, though a small amount of clear sputum may occur in rare cases.
Fatigue and Reduced Physical Stamina
Persistent fatigue due to decreased oxygen exchange efficiency.
Gradual decline in the ability to perform daily activities.
Chest Pain or Discomfort
Some patients may experience chest tightness or dull pain, especially during deep breaths.
Unexplained Weight Loss
A result of chronic inflammation or disease progression.
Cyanosis
Bluish discolouration of the lips and nail beds is caused by severe oxygen deprivation.
Clubbing of Fingers or Toes
Thickening and broadening of fingertips or toes due to prolonged hypoxia are commonly seen in advanced stages of the disease.

Causes

The causes of ILD are diverse and generally classified into two categories: known causes and idiopathic (unknown causes).

Known Causes of ILD:

Environmental and Occupational Exposures: Prolonged inhalation of harmful substances such as: Silica dust (Silicosis), Asbestos fibbers (Asbestosis), Coal mine dust or agricultural dust. These exposures can lead to chronic lung damage and fibrosis.
Drug-Induced: Certain medications may cause lung toxicity, such as: Chemotherapy drugs (e.g., bleomycin), Antibiotics (e.g., nitrofurantoin), Anti-inflammatory drugs (e.g., methotrexate)
Infections: Viral, bacterial, or fungal infections may lead to chronic inflammation, eventually damaging lung tissue.
Radiation Therapy: Radiation treatment for cancers such as lung or breast cancer can result in radiation pneumonitis or radiation-induced fibrosis.

Idiopathic Interstitial Pneumonias (IIPs):

Idiopathic Pulmonary Fibrosis (IPF): The most common form of IIP. While the exact cause is unknown, genetic and environmental factors are believed to play a role.
ILD Associated with Connective Tissue Diseases: Often linked with autoimmune conditions such as: Rheumatoid, arthritis, Systemic sclerosis (Scleroderma), Sjogren's syndrome, Systemic lupus erythematosus (SLE)
Hypersensitivity Pneumonitis (HP): It is caused by prolonged exposure to specific antigens (e.g., bird droppings, mould), resulting in an immune response that damages lung tissue.
Genetic Interstitial Lung Diseases: Mutations in specific genes, such as TERT (telomerase reverse transcriptase), may increase the risk of developing ILD.

Diagnosis

The diagnosis of interstitial lung disease (ILD) involves a combination of clinical evaluation, imaging studies, pulmonary function tests, and pathological analysis:

Clinical Examination	Assess symptoms (e.g., chronic cough, progressive dyspnoea), environmental exposure history, and medication history. Lung auscultation may reveal "Velcro crackles."
Imaging Studies	Chest X-ray: An initial screening tool that may show reticular or honeycomb patterns but has low sensitivity. High-Resolution Chest CT (HRCT): The gold standard for diagnosing ILD, providing detailed visualization of interstitial changes such as reticular fibrosis or honeycombing. Positron Emission Tomography-Computed Tomography (PET-CT): Useful for evaluating tumour-related causes.
Pulmonary Function Testing	Evaluates lung volumes and gas exchange function. ILD patients typically exhibit restrictive ventilatory defects and reduced diffusing capacity for carbon monoxide (DLCO).
Blood Tests	Assess for autoimmune diseases (e.g., antinuclear antibody [ANA], rheumatoid factor [RF], antineutrophil cytoplasmic antibody [ANCA]). Measure inflammatory markers such as C-reactive protein (CRP) or erythrocyte sedimentation rate (ESR).
Bronchoscopy and Bronchoalveolar Lavage (BAL)	Collect fluid samples from the trachea and alveoli to assess for inflammation or infectious pathogens.
Cryobiopsy	A minimally invasive method for tissue sampling using bronchoscopic guidance and cryotechnology, with these features: Tissue is "frozen" with a cryoprobe and then extracted. Provides larger samples for pathological analysis. Relatively low trauma can be performed during bronchoscopy.
Surgical Lung Biopsy	Performed via thoracoscopy when necessary to confirm the degree of fibrosis through histopathological examination.
Six-Minute Walk Test (6MWT)	Assesses exercise tolerance and changes in oxygen saturation during physical activity.

Treatments

The specific treatment approach depends on the underlying cause, severity, and the patient's overall health condition.

Pharmacological Treatment	Drug therapy primarily aims to suppress inflammation and slow fibrosis progression. The main categories of drugs include: Anti-fibrotic drugs Corticosteroids Immunosuppressants Biological agents and targeted therapies
Supportive Therapy	Even when a cure is not possible, supportive treatments can improve the quality of life and alleviate symptoms. (1) Oxygen Therapy Suitable for patients with hypoxemia to ease breathlessness and increase exercise tolerance. Use of home oxygen machines or portable oxygen devices during daily activities. (2) Pulmonary Rehabilitation A comprehensive program that includes: Exercise training: Strengthens respiratory muscles and improves cardiovascular fitness. Breathing techniques: Techniques such as pursed-lip breathing to relieve breathlessness. Psychological support: Helps patients cope with anxiety and depression, enhancing quality of life. Nutritional counselling: High-protein, high-energy diets to maintain body weight. (3) Vaccination Vaccines for influenza and pneumococcus reduce the risk of respiratory infections and prevent disease exacerbation.
Surgical Treatment	Recommended for patients with severe lung damage or poor response to medications. (1) Lung Transplantation Indications: End-stage ILD, particularly idiopathic pulmonary fibrosis. Goal: Restore respiratory function through the transplantation of healthy lungs. Considerations: Patient age, overall health status, and donor compatibility. (2) Video-assisted thoracoscopic Surgery (VATS) Used for diagnostic purposes to confirm ILD or assess disease progression. While primarily diagnostic, some patients experience symptom relief.
Lifestyle Modifications and Care	Daily management strategies can help control the disease and improve quality of life: Smoking cessation: Avoid smoking and exposure to second-hand smoke to reduce lung damage. Environmental precautions: Limit exposure to dust, mould, chemicals, or other allergens and toxins. Moderate exercise: Walking or swimming can improve lung and heart function. Regular follow-up: Monitor lung function, imaging changes, and medication side effects.