L07: Interstitial Lung Diseases Flashcards
What does the term interstitial lung diseases mean
Large groups of diseases that cause scarring (fibrosis) of the lungs
How does pulmonary fibrosis and start and develop
Lungs have inflammation which later scar and shrink
In a spirometry what does pulmonary fibrosis show
Low FVC Normal normal/high FEV1/FVC Low residual volume Low total lung capacity Low DLCO
What does gas exchange require for it to occur
Alveolar ventilation
Pulmonary circulation
Functioning alveoli
In pulmonary fibrosis what requirement of gas exchange becomes displaced
Functioning alveoli
Where can impairment come from
Impaired ventilation
Impaired circulation
Abnormal alveoli
What is pulmonary fibrosis
Scarring of the lung parenchyma usually after inflammation
What are the causes of inflammation that drives widespread pulmonary fibrosis
Drugs
Carcinomatosis
What are the causes of inflammation that drives upper pulmonary fibrosis
TB
Ankylosing spondylitis
Sarcoidosis
EAA
What are the causes of inflammation that drives lower pulmonary fibrosis
Cryptogenic
Connective tissue disease e.g rheumatoid arthritis
Occupational e.g asbestos
What does cryptogenic mean
Unknown cause
What are the clinical features of pulmonary fibrosis
Dry cough Dyspnoea (breathlessness) Fine end inspiratory creeps at base of lungs Middle age Mostly male
On a chest x-ray how does pulmonary fibrosis show
Reticular shadowing
Honeycombing
After an chest x-ray if there are features of a disease which diagnostic tool is used for more detail
High CT scan
What features are present on a high CT scan with pulmonary fibrosis
Subpleural reticular shadows
Honeycombing
If a diagnosis is not established after an high CT scan what diagnostic method tool should be used
Lung biopsy
What other features on a high CT scan show with pulmonary fibrosis
Localised fibrosis (fibrosis on one particular area) Ground glass change (glassy appearance)
What does a ground glass change indicate
Early active inflammation which can be reversed
What does management of pulmonary fibrosis depend on
Cause
Histology
Severity
Progression
What is the treatment for usual interstitial pneumonitis
Pirfenidone
Nintendanib
What is the treatment for autoimmune disease causing pulmonary fibrosis
Immunosuppression
What is the last treatment for pulmonary fibrosis
Lung transplantation
When is biopsy carried out
Only when the diagnosis is not clear
What is usual interstitial pneumonitis look like on a clinical image
Basal Subplerual Not much ground glass Honeycombing Nil on antibodies
