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Interstitial Lung Diseases

Respiratory MedicineCardio Thoracic Surgery

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.

Diagnosis and Treaments

Histologic Confirmation by Cryobiopsy

Cryobiopsy allows for the extraction of larger tissue specimens with higher quality. It results in less damage to the internal tissue structure of the sample, fully meeting the diagnostic requirements of pathologists while also causing less trauma during the procedure.

Diagnosis involves evaluating medical history, medications, exposure to harmful substances, and related symptoms. Tests like blood tests, chest X-rays, CT scans, electrocardiograms, and pulmonary function tests are commonly performed. Additional procedures like echocardiograms, bronchoscopies, or lung biopsies may be necessary in specific cases. Treatment depends on the underlying cause and symptom severity. Approaches include addressing infections, discontinuing causative medications, supplemental oxygen, steroid medications to reduce inflammation, drugs to slow lung damage, and pulmonary rehabilitation. Severe cases may require lung transplantation to replace damaged lungs with healthy ones.

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.

Factors

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.