Restrictive Lung Diseases Overview Flashcards
What three things can cause muscle weakness - intercostal or diaphragmatic - which causes thoracic restriction due to causes outwith the lungs?
Myopathy
Neuropathy
Myelopathy
What do Myaesthenia gravis, Guillan Barre, Motor neurone disease and poliomyelitis all cause, which leads to thoracic restriction of teh lungs?
Muscle weakness
How can abdominal obesity/ascites cause thoracic restriction to the lungs?
Compression of thoracic contents
What does thoracic restriction due to causes out with the lungs result in?
Chronic alveolar under ventilation with low PaO2 (decreased SaO2) and raised PaCO2 and reduced lung volumes
Name 5 causes within the lungs that cause thoracic restriction?
- Acute DPLD - diffuse parenchymal lung disease
- Episodic DPLP, all of which may present acutely
- Chronic DPLD due to occupational or environmental agents
- Chronic DPLD with evidence of systemic disease
- Chronic DPLD with no evidence of systemic disease
What is lung parenchyma?
Alveolar walls/lumen
What is the pathophysiology of restrictive thoracic disease due to diseases within the lung i.e. DPLD?
Imparied alveolar gas exchange - alveolar barrier to O2 exchange, CO2 exchange umimparied as alveolar ventilation normal
Leads to decreased PaO2 (decreased SaO2) and normal PaCO2
What can cause DPLD?
Fluid in the alveolar air spaces Consolidation of alveolar air spaces Inflammatory infiltrate of alveolar walls (alveolitis) Dust disease Carcinomatosis Eosinophilic
What two conditions can lead to fluid in the alveolar air sacs?
Cardiac pulmonary oedema (in alveolar walls and lumen)
Non-cardiac pulmonary oedema
What causes cardiac pulmonary oedema?
Raised pulmonary venoius pressure i.e. LVF
What causes non-cardiac pulmonary oedema?
Normal pulmonary venous pressure with leaky pulmonary capillaries - due to sepsis or trauma (ARDS aka shock lung) also due to altitude sickness
Give three causes of consolidation of alveolar air spaces?
Infective pneumonia
Infarction - PE/vasculitis
BOOP - Rheumatoid disease
Name an inflammatory infiltrate cause of alveolitis?
Granulomatous alveolitis
Give two granulomatous-alveolitis diseases?
Extrinsic allergic alveolitis
Sarcoidosis
What do Farmer’s lung and avian cause?
Extrinsic-Allergic-Alveolitis aka hypersensitivity pneumonitis- type III reaction
What is the name of a multi-system disease with clinical features such as lymphadenopathy, erythema nodosum, uveitis, myocarditis and neuropathy?
Sarcoidosis
What three drugs can induce alveolitis?
Amiodarone
Bleomycin
Methotrexate
What toxic gas fume can cause DPLD, alveolitis?
Chlorine
Give two features of fibrosing alveolitis?
Rheumatoid, cryptogenic
What classification of diseases do SLE, polyarteritis, Wegners, Churg-Straussand Bechet’s all come under?
Autoimmune (multi-systemic)
What is another term for dust disease?
Pneumoconiosis
What two subtypes are there to dust disease?
Fibrogenic and non-fibrogenic
Name 2 fibrogenic dust diseases?
Asbestosis
Silicosis
Name 3 non-fibrogenic dust-diseases?
Siderosis (iron)
Stanosis (Tin)
Baritosis (Barium)
What is nitrofurantoin?
Eosinophilic drug response
What is Aspergillosis?
Fungal eosinophilic response
What is Toxocara, Ascaris and Filaria?
Eosinophilic parasite response
What are Churg Strauss and Polyarteritis?
Autoimmune eosinophilic responses
What does pulmonary fibrosis occur as an end stage response to?
Chronic inflammation
Give 6 clinical features of DPLD?
- Breathless on exertion
- Cough but no wheeze
- Finger clubbing
- Inspiratory lung crackles
- Central cyanosis (if hypozaemic)
- Pulmonary fibrosis
What can be said about the lung volumes in the diagnosis of DPLD?
Reduced lung volumes:
Decreased FEV1
Decreased FVC normal ration > 75%
Peak flow is normal
What condition has reduced gas diffusion (DLCO) and arterial oxygen desaturation (decreased PaO2 and decreased SaO2)?
DPLD
What might be raised in sarcoidosis and so be used for diagnosis?
Serum ACE and Ca
In some DPLD what antibodies might you look for?
Avian, fungal, auto-antibodies (rheumatoid, anti-nuclear)
In DPLD will there be bilateral diffuse alveolar infiltrates on CXR?
Yes
In the diagnosis of DPLD, why would you do an echocardiogram?
To exclude LVF
What investigation can differentiate between inflammatory ground glass and fibrotic nodular component of alveolar infiltrates?
High resolution CT
In the diagnosis of DPLD - why mighy you do bronchoalveolar lavage or induced sputum?
To exclude pneumocysits and TB
If there is ground glass inflammation on HRCT, what would you treat with?
Immunosuppressives
What is the first line treatment of DPLD?
Systemic corticosteroids i.e. oral prednisolone
Can you use inhaled corticosteroids to treat DPLD?
No
What is the second line treatment for DPLD?
Oral azathioprine [steroid sparing]
Name an anti-fibrotic agent?
Pirfenidone
What would you use anti-fibrotic agents for?
Idiopathic pulmonary fibrosis
What would you use anti-oxidant agents for?
Acetylcysteine for Idiopathic Pulmonary Fibrosis
In what disease would you get bilateral hilar lymphadenopathy and lung infiltrates on CXR?
Sarcoidosis
What type of granulomas does sarcoidosis have?
Non caseating ggranuloma
What three skeletal related conditions can lead to thoracic restriction due to causes out with lungs?
- Thoracic kyphoscoliosis
- Ankylosing spondylitis
- Rib trauma