Restrictive Lung Disease Flashcards
what is the main pathology behind restrictive lung disease?
alveolar barrier to O2 exchange
describe PaO2 and PaCO2 in restrictive lung disease?
low PaO2
normal PaCO2
what are the 2 types of pulmonary oedema?
cardiac pulmonary oedema
non cardiac pulmonary oedema
why does cardiac pulmonary oedema occur?
due to raised pulmonary pressure in the veins due to back pressure from the heart
why does non cardiac pulmonary oedema occur?
leaky pulmonary capillaries (can be caused by sepsis or trauma- ARDS)
what are the 3 major causes of consolidation of the alveolar air spaces?
infective pneuonia
infarction
others
what 2 types of infarction can occur in the lung and thus cause consolidation of the alveolar air?
pulmonary embolism
vasculitis
what is the name of the non-infectious type of pneumonia which causes alveolar air space consolidation?
cryptogenic organizing pneumonia
what disease is associated with cryptogenic organising pneumonia?
rheumatoid disease
what anti-arrhythmic medication is associated with cryptogenic organising pneumonia?
amiodarone
what type of hypersensitivity reaction is extrinsic allergic alveolitis?
type 3: immune-complex mediated
hypersensitivity pneumonitis
what type of hypersensitivity reaction is sarcoidosis?
type 4:delayed hypersensitivity
what 5 important signs can be caused by sarcoidosis?
- lymphadenopathy
- erythema nodosum
- uveitis
- myocarditis
- neuropathy
what are the 2 causes of fibrosing alveolitis?
rheumatoid associations
cryptogenic fibrosing alveolitis
what 6 important diseases cause an inflammatory infiltrate of the alveoler walls (alveolitis) and so cause restrictive disease?
- extrinsic allergic alveolitis
- sarcoidosis
- drug induced alveolitis
- pneumoconiosis
- fibrosing alveolitis
- autoimmune alveolitis (eg SLE)
what is pneumoconiosis?
dust-disease
what two types of pneumoconiosis is there?
fibrogenic
non fibrogenic
what are the 2 important types of fibrogenic pneumoconiosis?
asbestosis
silicosis
what are 3 important types of non-fibrogenic pneumoconiosis?
siderosis (iron)
stanosis (tin)
baritosis (barium)
what are the 6 important features of the clinical syndrome of restrictive lung disease?
- breathless on exertion
- cough (with no wheeze)
- finger clubbing
- inspiratroy lung crackles
- central cyanosis
- pulmonary fibrosis (late stage)
why does pulmonary fibrosis occur in late stage restrictive disease?
as a response to the chronic inflammation
what are 4 key questions you must ask to find out the cause of restrictive disease?
occupation?
medications?
pets?
arthritis?
in restrictive disease, what is the DLCO?
reduced
in restrictive disease what is the PEFR like?
normal
in restrictive disease what is FEV1, FVC and FEV1/FVC like?
reduced FEV1
reduced FVC
normal ratio
in sarcoidosis, what 3 findings would you expect when testing serum?
raised ACE
raised Ca
increased inflammatory markers
why do you use an echocardiogram in diagnosing restrictive lung disease?
to rule out LVF
how do you treat restrictive lung disease?
- remove trigger (eg dust, drug, allergen)
- treat any inflammation with immunosupressives
- give oxygen if hypoxaemia
- lung trasplant
what is the 1st line treatment for restrictive lung disease?
systemic corticosteroids
eg oral prednisolone
what is the 2nd line treatment for restrictive lung disease?
oral azathioprine (a steroid sparing, immunosuppressive)
what anti-fibrotic agent can be used in some restrictive lung disease?
pirfenidone
what anti-oxidant agents can be used in some restrictive lung disease?
acetylcysteine
what is the type of infiltrate formed in sarcoidosis?
non caseating granuloma (T cell and macrophage infiltrate)
what are the 3 types of interstitial lung disease? (in relation to timing/onset(
- acute
- episodic
- chronic (most common)
what are the 4 types of interstitial lung disease?
- ILD of known cause
- idiopathic interstitial pneumonia
- granulomatous ILDs
- others
how can sarcoidosis affect the kidneys directly?
granulomas in the kidneys
how can sarcoidosis affect the kidneys indirectly?
due to increased calcium
what 2 types of sarcoidosis can occur?
acute or chronic
what do you see on a CXR or CT scan of the lungs in a patient with sarcoidosis?
bilateral hilar lymphadnopathy
peripheral nodular infiltrate
what do you see on a tissue biopsy of a patient with sarcoidosis?
non-caseating granuloma
what is the treatment of acute sarcoidosis?
self-limiting, usually no treatment
steroids if vital organ infected
what is the treatment of chronic sarcoidosis?
oral steroids usually needed, immunosuppression (eg azathioprine, methotrexate, anti-TNF therapy)
what do you use the raised ACE levels to measure in sarcoidosis?
marker of improvement
not a diagnostic test
how long do you monitor patients for after an acute episode of sarcoidosis?
1 year
what drug can cause extrinsic allergic alveiolitis?
belomycin (chemotherapy drug)
when do you get symptoms of extrinsic allergic alveolitis?
several hours after acute exposure
what is seen on an CXR of a patient having acute extrinsic allergic alveolitis?
widespread pulmonary infiltrates
what is the treatment of acute extrinsic allergic alveolitis?
oxygen
steroids
antigen avoidance
what is the cause of chronic extrinsic allergic alveolitis?
repeated low dose antigen exposure over time causing scarring resulting in progressive breathlessness and cough
what would be the may clinical sign on chest examination for a patient with chronis extrinsic allergic alveolitis?
crackles
what would you see in a CXR of a patient with chronic extrinsic allergic alveolitis?
pulmonary fibrosis (Scarring), most commonly in ther upper zones
what is the treatment of chronic extrinsic allergic alveolitis?
antigen avoidance
oral steroids
how is fibrosis shown on CXR?
honey-combing
what is the most common interstitial lung disease?
idiopathic pulmonary fibrosis (cryptigenic fibrosins alveolitis)
what is the pathogenesis of idiopathic pulmonary fibrosis?
imbalance in fibrotic repair system,
not an inflammatory disease
what signs would you see on respiratroy examination of a patient with idiopathic pulmonary fibrosis?
clubbing
inspiratory crackles
what drug is the only useful drug in idiopathic pulmonary fibrosis?
pirfenidone
what is simple pneumoconiosis?
abnormalitiy on CXR only, no impairement of lung function
what is complicated pneumoconiosis?
progressive massive fibrosis causing a restrictive pattern of lung disease
can cause SOB, haemoptysis, respiratroy failure
what is caplans syndrome?
a combination of rheumatoid arthritis and pneumoconiosis that manifests as pulmonary nodules
in simple pneumoconiosis what is seen on an CXR??
lots of little dots
in complicated pneumoconiosis what is seen on a CXR?
balls of fibrous tissue
what 3 categories of disease can you get from asbestos exposure?
pleural disease
pulmonary fibrosis
bronchial carcinoma
what 4 types of pleural disease are related to asbestos exposure?
- benign pleural plaques
- acute asbestos pleuritis
- pleural effusion and diffuse pleural thickening
- malignant mesothelioma
what are the symptoms for benign pleural plaques?
asymptomatic
what are the symptoms for acute asbestos pleuritis?
fever, pain ,bloody pleural effusion
what are the symptoms for pleural effusion and diffuse pleural thickening?
restrictive impairement
what is the name of pulmonary fibrosis related to asbestos exposire?
asbestosis
a type of pneumoconiosis
what characterises the early stage of interstitial lung disease?
alveolitis (injury with inflammatory cell infiltration)
what characterises the late stage of lung disease?
fibrosis
what type of lung biopsies can you take?
transbronchial biopsy (during bronchoscopy) thoracoscopic biopsy (minor thoracic surgery)
what are the pros and cons of a thoracoscopic biopsy?
more relieble
generates far more tissue
more invasive
(compared to a transbronchial biopsy)
what are the 2 types of asbestos fibres?
serpentine (curved)
straight
which type of asbestos is safer?
serpentine asbestos, gets stuck in larger airways so cant get into alveoli
