Interstitial Lung Disease + Sarcoidosis Flashcards
What are Interstitial lung diseases?
Collective term for a group of disorders that cause inflammation and fibrosis disrupting the interstitium and the parenchyma of the lungs
How do patients with interstitial lung diseases typically present?
Chronic:
-SOB
-dry/non productive cough
-fatigue
Dry Inspiratory crackles
Finger clubbing
Chest pain
Weight loss?
What are the 2 main types of Interstitial Lung Disease?
Primary ILD
Secondary ILD
What is primary interstitial Lung disease?
Idiopathic/not caused by anything else
What is secondary interstitial lung disease?
When the interstitial lung disease is caused by something else
What is the most common cause of primary interstitial lung diseases?
Idiopathic pulmonary fibrosis / idiopathic interstitial pneumonia
What are some examples to ask a patient you think might have an interstitial lung disease?
SOB?
Cough non-productive?
Chest pain?
Occupational history
Family history and PMH
Acute on chronic? (Infectious signs)
What are some medications that can cause secondary interstitial lung disease?
Amiodarone
Bleomycin
Nitrofurantoin
What are some occupational causes of interstitial lung diseases?
Pneumoconiosis
Silicosis
Asbestosis
Byssinosis (cotton)
Hypersensitivity pneumonitis:
-bird fanciers lung
-cheese workers lung
What pre-existing medical conditions can cause pulmonary fibrosis?
Rheumatoid arthritis
Systemic sclerosis
Idiopathic myositis
Primary Sjögren’s syndrome
Mixed connective tissue disease
Sarcoidosis
Alpha-1-anti trypsin deficiency
What first line investigations would you do for a patient presenting with chronic SOB, chest pain and a dryer productive cough? (?ILD)
Routine bloods
ABG
O2 sats
6min walk tests
ECG
CXR
When you are then suspecting interstitial lung disease after doing your first line investigations, what further investigations will you do to help diagnose?
HRCT
Pulmonary function tests/Spirometryy
Echocardiography (pulmonary HTN)
Bronchoscope+- biopsy
Autoantibody screen (ANA, anti-Scl-70, rheumatoid factor, anti ro, anti la)
Bronchioalveolar lavage
Lung biopsy
What is expected to be seen on a HRCT of a patient with interstitial lung disease?
Bilateral bi-basal reticular shadowing
Honey combing
Ground glass opacity for idiopathic pulmonary fibrosis
Mediastinal lymphadenopathy
What type of result would you expect to see on pulmonary function tests/spirometry for an interstitial lung disease?
Restrictive curve (wizard hat) NOT SCALLOPING, that’s for obstructive
FEV1:FVC ratio above 0.7
How do you manage an interstitial lung disease?
Oxygen therapy
Cease smoking
Avoid cause
Treat autoimmune condition
Physiotherapist
Transplant if severe and young patient
What types of medications do you give to treat/manage idiopathic pulmonary fibrosis?
Antifibrotic agents
What are some anti-fibrotic medications you can give to slow the progression of idiopathic pulmonary fibrosis?
Perfenidone
Nintedanib
Both reduce fibrosis and inflammation.
What is the pathophysiology of hypersensitivity pneumonitis?
Extrinsic allergic alveolitis involving type III and type IV hypersensitivity reactions to inhaled allergens
How is a hypersensitivity pneumonitis diagnosed?
Bronchoalveolar lavage contains lymphocytes