Interstitial Lung Disease Flashcards
What is interstitial lung disease (ILD)?
ILD refers to a group of disorders characterised by inflammation and fibrosis of the lung interstitium, impairing gas exchange.
What are the common types of ILD?
Common types include idiopathic pulmonary fibrosis (IPF), hypersensitivity pneumonitis, sarcoidosis, and connective tissue disease-associated ILD.
What are the symptoms of ILD?
Symptoms include progressive dyspnoea, chronic dry cough, fatigue, and, in advanced cases, cyanosis.
What are the signs of ILD on clinical examination?
Signs include bibasal fine crackles, finger clubbing, and in advanced cases, signs of pulmonary hypertension or right heart failure.
What is the pathophysiology of ILD?
ILD involves inflammation and fibrosis of the lung interstitium, leading to thickened alveolar walls and impaired oxygen diffusion.
What are the common causes of ILD?
Causes include idiopathic conditions (e.g., idiopathic pulmonary fibrosis), environmental exposures, autoimmune diseases, infections, and certain medications.
What are the risk factors for ILD?
Risk factors include smoking, occupational exposures (e.g., asbestos, silica), genetic predisposition, and underlying autoimmune disease.
What are the key features of idiopathic pulmonary fibrosis (IPF)?
IPF is characterised by progressive dyspnoea, bibasal crackles, and honeycombing on imaging, typically affecting older adults.
What environmental exposures can cause ILD?
Exposures include asbestos (asbestosis), silica (silicosis), bird droppings, and moulds (hypersensitivity pneumonitis).
What autoimmune conditions are associated with ILD?
Conditions include rheumatoid arthritis, systemic sclerosis, Sjögren’s syndrome, and dermatomyositis.
How is ILD diagnosed?
Diagnosis is based on clinical history, physical examination, pulmonary function tests, imaging (e.g., HRCT), and sometimes lung biopsy.
What are the pulmonary function test findings in ILD?
PFTs typically show a restrictive pattern with reduced FVC, TLC, and DLCO, but normal or increased FEV1/FVC ratio.
What are the key high-resolution CT (HRCT) findings in ILD?
Findings include ground-glass opacities, honeycombing, and reticular patterns depending on the underlying cause.
What blood tests are used to investigate ILD?
Blood tests include autoimmune panels (e.g., ANA, RF), serum ACE for sarcoidosis, and inflammatory markers.
What is the role of bronchoalveolar lavage (BAL) in ILD?
BAL helps identify inflammatory or infectious causes of ILD and can provide clues about environmental exposures.
What is the role of lung biopsy in ILD?
Lung biopsy is used when the diagnosis is unclear and may provide definitive histological findings.
What are the treatment options for idiopathic pulmonary fibrosis (IPF)?
Treatments include antifibrotic agents like pirfenidone or nintedanib and supportive care such as oxygen therapy and pulmonary rehabilitation.
What is the management of hypersensitivity pneumonitis?
Management includes avoiding the triggering antigen and, in some cases, corticosteroids.
What medications are associated with ILD?
Medications include amiodarone, methotrexate, nitrofurantoin, and certain chemotherapy agents like bleomycin.
What complications can arise from ILD?
Complications include pulmonary hypertension, respiratory failure, right heart failure, and increased risk of lung cancer.
What lifestyle modifications are recommended for ILD patients?
Recommendations include smoking cessation, avoiding environmental triggers, regular exercise, and receiving vaccinations (e.g., influenza, pneumococcus).
What is the prognosis for ILD?
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.
What is the role of lung transplantation in ILD?
Lung transplantation may be considered for severe, refractory cases of ILD, particularly IPF.
What is the differential diagnosis for ILD?
Differential diagnoses include COPD, heart failure, pulmonary embolism, and lung infections.
What are the features of sarcoidosis-associated ILD?
Features include hilar lymphadenopathy, non-caseating granulomas, and multisystem involvement, such as skin and eye lesions.
