Interstitial Lung Disease

Question 1

What is interstitial lung disease (ILD)?

Answer 1

ILD refers to a group of disorders characterised by inflammation and fibrosis of the lung interstitium, impairing gas exchange.

Question 2

What are the common types of ILD?

Answer 2

Common types include idiopathic pulmonary fibrosis (IPF), hypersensitivity pneumonitis, sarcoidosis, and connective tissue disease-associated ILD.

Question 3

What are the symptoms of ILD?

Answer 3

Symptoms include progressive dyspnoea, chronic dry cough, fatigue, and, in advanced cases, cyanosis.

Question 4

What are the signs of ILD on clinical examination?

Answer 4

Signs include bibasal fine crackles, finger clubbing, and in advanced cases, signs of pulmonary hypertension or right heart failure.

Question 5

What is the pathophysiology of ILD?

Answer 5

ILD involves inflammation and fibrosis of the lung interstitium, leading to thickened alveolar walls and impaired oxygen diffusion.

Question 6

What are the common causes of ILD?

Answer 6

Causes include idiopathic conditions (e.g., idiopathic pulmonary fibrosis), environmental exposures, autoimmune diseases, infections, and certain medications.

Question 7

What are the risk factors for ILD?

Answer 7

Risk factors include smoking, occupational exposures (e.g., asbestos, silica), genetic predisposition, and underlying autoimmune disease.

Question 8

What are the key features of idiopathic pulmonary fibrosis (IPF)?

Answer 8

IPF is characterised by progressive dyspnoea, bibasal crackles, and honeycombing on imaging, typically affecting older adults.

Question 9

What environmental exposures can cause ILD?

Answer 9

Exposures include asbestos (asbestosis), silica (silicosis), bird droppings, and moulds (hypersensitivity pneumonitis).

Question 10

What autoimmune conditions are associated with ILD?

Answer 10

Conditions include rheumatoid arthritis, systemic sclerosis, Sjögren’s syndrome, and dermatomyositis.

Question 11

How is ILD diagnosed?

Answer 11

Diagnosis is based on clinical history, physical examination, pulmonary function tests, imaging (e.g., HRCT), and sometimes lung biopsy.

Question 12

What are the pulmonary function test findings in ILD?

Answer 12

PFTs typically show a restrictive pattern with reduced FVC, TLC, and DLCO, but normal or increased FEV1/FVC ratio.

Question 13

What are the key high-resolution CT (HRCT) findings in ILD?

Answer 13

Findings include ground-glass opacities, honeycombing, and reticular patterns depending on the underlying cause.

Question 14

What blood tests are used to investigate ILD?

Answer 14

Blood tests include autoimmune panels (e.g., ANA, RF), serum ACE for sarcoidosis, and inflammatory markers.

Question 15

What is the role of bronchoalveolar lavage (BAL) in ILD?

Answer 15

BAL helps identify inflammatory or infectious causes of ILD and can provide clues about environmental exposures.

Question 16

What is the role of lung biopsy in ILD?

Answer 16

Lung biopsy is used when the diagnosis is unclear and may provide definitive histological findings.

Question 17

What are the treatment options for idiopathic pulmonary fibrosis (IPF)?

Answer 17

Treatments include antifibrotic agents like pirfenidone or nintedanib and supportive care such as oxygen therapy and pulmonary rehabilitation.

Question 18

What is the management of hypersensitivity pneumonitis?

Answer 18

Management includes avoiding the triggering antigen and, in some cases, corticosteroids.

Question 19

What medications are associated with ILD?

Answer 19

Medications include amiodarone, methotrexate, nitrofurantoin, and certain chemotherapy agents like bleomycin.

Question 20

What complications can arise from ILD?

Answer 20

Complications include pulmonary hypertension, respiratory failure, right heart failure, and increased risk of lung cancer.

Question 21

What lifestyle modifications are recommended for ILD patients?

Answer 21

Recommendations include smoking cessation, avoiding environmental triggers, regular exercise, and receiving vaccinations (e.g., influenza, pneumococcus).

Question 22

What is the prognosis for ILD?

Answer 22

Prognosis varies by type; idiopathic pulmonary fibrosis has a poor prognosis with a median survival of 3–5 years, while other types may stabilise with treatment.

Question 23

What is the role of lung transplantation in ILD?

Answer 23

Lung transplantation may be considered for severe, refractory cases of ILD, particularly IPF.

Question 24

What is the differential diagnosis for ILD?

Answer 24

Differential diagnoses include COPD, heart failure, pulmonary embolism, and lung infections.

Question 25

What are the features of sarcoidosis-associated ILD?

Answer 25

Features include hilar lymphadenopathy, non-caseating granulomas, and multisystem involvement, such as skin and eye lesions.