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
What is non specific interstitial pneumonitis
Anything non-specific that does not look like usual interstitial pneumonitis
What are the other rarer interstitial lung disease that may cause fibrosis
Extrinsic allergic alveolitis
Granulomatous lung disease
What is extrinsic allergic alveolitis (EAA)
Caused by inhalation of organic particles that causes hypersensitivity response leading to scarring of the lungs
What are the 3 ways that EAA can present
Acute
Chronic
Sub-acute
How do we diagnose EAA
Identify the organic particle from patient history
Chest-X-ray
High CT
Blood
What does a chest x-ray and high CT scan show with EAA
Upper lobe fibrosis
What does the blood sample of EAA show
Positive avian precipitins
Normal IgE
What is the treatment of EAA
Get rid of antigen such as birds
Steroid
What does the term granulomatosus lung disease mean
Group of infectious and non infections characterised by the formation of granulomas
What are the 3 sub causes of granulomatous lung disease
Bugs
Inflammatory
Vasculitic
What is sarcoidosis
A multi-system disease with unknown cause with non caseating seating granulomas
How does sarcoidosis affect the skin
Via lofgrens syndrome
What does lofgrens syndrome present with
BHL
Erythema nodes
Polyarthralgia
What other areas can sarcoidosis affect on the body outside the lungs
Skin Eyes Heart Neuro Liver
How do we investigate sarcoidosis
Full lung function test such as spirometry
Hight CT
ECG
Blood test of ACE
What is the treatment of sarcoidosis
Steroids
What is vasculitis
Inflammation of blood vessels
Name an example of vasculitis
Wegeners granulomatosis
What do you see with wegeners in the upper airway
Granulomas that lead to bleeding
Sinusitis
Saddle nose
What do you see with wegeners in the lungs
Haemorrhage
Cavities
What is the FEV1/FVC ratio
The ratio of the forced expiratory volume in the first second (rate)
What is DLCO
A measurement that asses the ability of gas exchange in the alveoli
