Restrictive, Mixed Restrictive and Obstructive Lung Disease Flashcards
What is restrictive lung disease?
- inability to inhale - ⬇️ compliance of lungs
When doing a spirometry test in patients with restrictive lung disease what would you expect to see in FVC, FEV1 and the FEV1/FVC ratio?
- ⬇️ FVC
- normal or ⬇️ FEV1 (relative to their own FVC
- ⬆️ FEV1/FVC ratio (due to FVC)
- <70% FEV1/FVC ratio is diagnostic
In patients with respiratory lung disease we would expect to see reductions in: - inspiratory reserve volume - tidal volume - expiratory reserve volume - residual volume Why is this the case?
- inability to inflate lungs (reduced compliance)
- lungs appear smaller (seen on volume flow loops)
- observable on X-ray
What is parenchymal lung disease?
- disease affecting the functioning cells of the lungs
Specifically, where in the lungs does parenchymal lung disease generally affect?
- interstitium - space around alveoli
Pulmonary fibrosis is a restrictive lung disease, specifically parenchymal lung disease, what does this do to the interstitium?
- interstitium becomes inflamed and swollen
- interstitium becomes fibrotic
- ⬇️ compliance in interstitium
What is sarcoidosis as a parenchymal lung disease (restrictive lung disease)?
- accumulation of inflammatory cells
- accumulation of matrix proteins
- formation of granulomas generally at hilar lymph nodes
- multisystem
What is pneumoconiosis as a parenchymal lung disease (restrictive lung disease)?
- dangerous particles enter and damage lungs
- commonly called an occupational lung disease
What does pneumoconiosis mean?
- name comes from greek
- pneumo = wind or breathe (also pneumocytes)
- cono = dust
- isis = inflammation
Pleural effusions can turn into a pleural disease, which are forms of respiratory disease, what is a pleural effusion?
- ⬆️ fluid in pleural space
Pneumothorax can turn into a pleural disease, which are forms of respiratory disease, what is a pneumothorax?
- collapsed lung
- caused by air leaking into pleural space
- ⬇️ volume = ⬆️ pressure on lung
Haemothorax can turn into a pleural disease, which are forms of respiratory disease, what is a haemothorax?
- accumulation of blood in pleural space
Pleural calcification/thickening can turn into a pleural disease, which are forms of respiratory disease, what is pleural calcification/thickening?
- calcification of pleural walls
- causes thickening and stiffening of pleura
- can be caused by asbestos (pleural plaques)
Why can obesity be classified as a restrictive lung disease?
- fat compressed thoracic walls
- diaphragm and lungs cannot inhale
What are some common diseases that affect the chest wall and ultimately reduce total lung capacity that are classed as restrictive lung diseases?
- neuromuscular
- diaphragmatic palsy
- kyphosis
- scoliosis
If the parenchyme of the lungs is damaged, what does this commonly cause?
- inflammation
- fibrosis follows
In diffused parenchymal lung disease (restrictive lung disease), what happens to fibroblasts?
- migration and proliferation
- at site of damage
- leads to fibrotic loci
What are the 3 main diffused parenchymal lung disease (restrictive lung disease) that we are required to know about?
1 - Sarcoidosis
2 - Idiopathic Interstitial Pneumonia
3 - Non-Specific Interstitial Pneumonia
If the interstitium becomes damaged, inflamed and fibrotic, what does that do to the lungs ability to perfuse O2?
- O2 perfusion is ⬇️
- SAO2 is ⬇️
- patients become breathless
When taking a history of patients with suspected lung disease, what are some of the most common things that should be included?
- occupational history
- pets, specifically birds
- drug use
- radiation exposure
- autoimmune disorders
- HIV
- family history
What are the 4 most common symptoms observed in patients with diffused parenchymal lung disease, a restrictive lung disease?
- breathlessness (progressively worsens)
- cough
- fatigue (low Sa02)
- weight loss
In specific autoimmune associated diffused parenchymal lung disease, a restrictive lung disease, what are some additional common symptoms that they may present with?
- difficulty swallowing
- cold hands
- joint pain
- weight loss
- skin rash
When examining a patient with suspected diffused parenchymal lung disease, a restrictive lung disease, what may there respiratory rate be?
- ⬆️ respiratory rate
- tachypnoea or dysponea
What is tachypnoea?
- rapid short breathing
- normal respiratory rate is 12-16 breaths/minute
What is dysponea?
- greek for disordered breathing
- shortness of breathe
Clubbing can be common in patient with suspected diffused parenchymal lung disease, a restrictive lung disease, what is clubbing?
- thickening of tips of toes and fingers
- look at fingers side on
- feel fingers for swelling
- loss of schamroth angle between fingers
What lung sounds may be heard in patient with suspected diffused parenchymal lung disease, a restrictive lung disease?
- fine crackles
- bibasal = bottom of both lungs
What cardiovascular changes may occur in patients with suspected diffused parenchymal lung disease, a restrictive lung disease?
- increased pressure in lungs back up into right side of heart
- RA pressure seen in ⬆️ jugular venous pressure
- ⬆️ peripheral oedema
- loud P2 sound (PV closing - pulmonary hypertension)
In patients with suspected diffused parenchymal lung disease, a restrictive lung disease what happens to SaO2, especially during exertion?
- ⬇️ SaO2
- ⬇️ SaO2 upon exertion
In addition to all the common presentations associated with diffused parenchymal lung disease, a restrictive lung disease, what may patients with an autoimmune form of the disease also present with?
- skin changes
- eye signs
- joint signs
What does idiopathic mean?
- no known cause
What is idiopathic pulmonary fibrosis?
- idiopathic = no known cause
- pulmonary = lungs
- fibrosis = excess collagen that becomes fibrotic
- lungs become damaged and inflamed
- lungs scar and become fibrotic, specifically in interstitium
What is the prevalence of idiopathic pulmonary fibrosis in the UK?
- 6-14.6 per 100,000
What age does idiopathic pulmonary fibrosis increase significantly?
- >75 year olds
Is idiopathic pulmonary fibrosis more common in patients above or below 50 years old?
- >50 years old
Is idiopathic pulmonary fibrosis more common in males or females?
- males
Why does idiopathic pulmonary fibrosis cause crackles on auscultation?
- alvelor air sacs popping open and collapsing
- sounds like when you dive and breathe
What is the prognosis in idiopathic pulmonary fibrosis?
- poor
- median survival is only 2.5-3.5 years
- exacerbations can kill, such as infections
On an X-ray or Ct scan, what may be present in patients with idiopathic pulmonary fibrosis?
- really small lungs
- honeycomb appears
How is idiopathic pulmonary fibrosis treated?
- no known cure
- symptoms (cough and hypoxia) are treated
- antifibrotic therapy
- lung transplant
- palliative care
What is non-specific interstitial pneumonia?
- form of idiopathic pulmonary fibrosis
- has been linked with autoimmune disease
What is collagen vascular disease?
- form of autoimmune disease
- body attacks its own collagen throughout the body
What is the median age of patients with non-specific interstitial pneumonia?
- 40-50 years old
- not linked to smoking
What symptoms are common in non-specific interstitial pneumonia?
- progressive worsening of shortness of breathe over 1 years
- cough
- crackles
- weight loss
- fatigue
What 3 diseases is non-specific interstitial pneumonia associated with?
1 - scleroderma
2 - rheumatoid arthritis
3 - systemic lupus erythematosus
Does non-specific interstitial pneumonia or idiopathic pulmonary fibrosis have a better prognosis?
- non-specific interstitial pneumonia
- better response to steroids
How is non-specific interstitial pneumonia treated?
- treat underlying cause
- immunotherapy
- long term O2 therapy
- palliative care
What causes sarcoidosis, a parenchymal lung disease (restrictive lung disease)?
- no known aetiology
- linked with autoimmune deficiency
- mainly affect the lungs
- may be genetic inheritance
Is sarcoidosis, a parenchymal lung disease (restrictive lung disease) permanent?
- no
- can resolve itself
- can remain latent and present at any time
- remains latent in granulomas of T cells and macrophages
Is sarcoidosis more common in men or women?
- women
What age group does sarcoidosis normally affect?
- 20-50 years old
Is there an ethnicity affect of sarcoidosis?
- yes
- Scandinavian, Afro-Caribbean and African American
What is the incidence of Sarcoidosis in the UK?
- 5 per 100,000
- most common idiopathic pulmonary fibrosis disease in UK
What infection of the lungs is Sarcoidosis commonly associated with?
- tuberculosis which has similiar symptoms
- also linked with lymphoma
What are the acute symptoms of sarcoidosis?
- presents similiar to the flu
- fever/night sweats
- arthralgia (joint pain)
- myalgia (muscle pain)
- anterior uveitis (inflammation of middle of the eye) - breathlessness
- ⬇️ appetite
- weight loss
- erythema nodosum
- lymphadenopathy
What are the 2 most common acute symptoms of sarcoidosis?
- erythema nodosum (painful red skin)
- anterior uveitis (painful red eyes)
How long do sarcoidosis symptoms last in an acute bout?
- short duration
- usually self limiting
How long do sarcoidosis symptoms last in an chronic bout?
- longer than acute
- radiological changes become present
- may see bilaterl lymphadenopathy
How is sarcoidosis commonly identified?
- generally an incidental finding
- best on a CXR
What might a patient with chronic sarcoidosis present with when sarcoidosis is active?
- progressive worsening of breathlessness
- ⬇️ exercise tolerance
- cough
- ⬇️ appetite/anorexia
- weight loss
Sarcoidosis can be multisystem, what organs can it affect?
- neurological symptoms
- bone pain
- skin lesions (nodules erythema nodosum)
- renal stones (due to hypercalcaemia)
What % of patients with sarcoidosis have any lung involvement, or only lung involvement?
- 50% have only lung involvement - 90% have some form of lung involvement
How can sarcoidosis be diagnosed?
- no definitive test
- combination of clinical experience and tests
- bilateral lymphadenopathy on CXR
- hypercalcaemia (created by macrophages)
- high T cells and ACE (produced by T cells)
- biopsy
What are some common tests used to help diagnose sarcoidosis?
- family and occupational history
- radiography (CXR)
- lung function
- ECG (arrythmias)
- blood and urine analysis
- eye examination
- skin test (rule out TB)
- angiotensin converting enzymes (ACE)
- hypercalcaemia
How many different stages of sarcoidosis are there?
- 5 stages - 0 - IV
Is sarcoidosis only a restrictive lung disease?
- no - can be obstructive and restrictive
Why is angiotensin converting enzymes (ACE) measured in patients with suspected sarcoidosis?
- T cells and macrophages are increased and can form granulomas
- both can secrete ACE
- so if sarcoidosis is active ACE wil be higher
What is the prognosis of sarcoidosis?
- 2/3 in remission within 10 years - those in stage 1, 60% in remission in 2 years - 1/3 progress to significant organ damage
What is the mortality rate in patients with sarcoidosis?
- 1-5% die - respiratory failure - cardiac arrhythmia - neurosarcoidosis
How are patients with sarcoidosis generally treated?
- oral prednisolone (steroid) - stage 2-4
- sarcoidosis and TB present similarly
- steroid worsen TB, so correct diagnosis is key!
Do asymptomatic or stage 1 sarcoidosis patients receive any specific treatment?
- no generally observation only
What is an exudate?
- think E for Exit incorrectly
- fluid from circulatory system
- generally due to inflammation
- high protein and lactate dehydrogenase content (similiar to blood)
What is the protein and lactate dehydrogenase (LDH) content of exudate?
- ⬆️ protein >0.5
- ⬆️ LDH >0.6
- 2/3 of serum levels (higher than serum)
What is lactate dehydrogenase?
- enzyme in all cells
- converts pyruvate to lactate
What is transudate?
- think T = transversing the membrane
- thinner fluid than exudate
- extravascular fluid
- generally due to high hydrostatic or low osmotic pressures
What is the protein and lactate dehydrogenase (LDH) content of transudate?
- ⬇️ protein <0.5
- ⬇️ LDH <0.6
- 2/3 of serum levels (higher than serum)
When could a pneunomthorax (air in pleural space) occur?
- generally due to trauma
- spontaneous symptoms
When could a haemomthorax (blood in pleural space) occur?
- generally due to trauma
- spontaneous symptoms
What are mesothelioma cells?
- simple squamous cells originating from mesoderm
- cells lining pleural walls
- secrete pleural fluid
What is a common cause of thickening of the pleura and mesothelioma?
- exposure to chemicals and toxins
- specifically asbestos
What is the only way to test if fluid in the pleural space is an exudate or transudate?
- pleural aspiration guided by ultrasound
- pleural drainage can be used if lots of fluid
When analysing pleural fluid what are the 3 types of tests that are required?
- biochemistry (protein and lactate dehydrogenase)
- microbiology (rule out TB)
- cytology (rule out cancer)
When investigating pleural aspirations what else is required to compare this with?
- serum blood sample
What is Lights Criteria?
- accurate diagnosis of pleural effusions
- determines if effusion is transudate or exudate
What are the 2 most common causes of transudate pleural effusions?
1 - increase hydrostatic pressure
- pulmonary hypertension and congestive cardiac failure
2 - ⬇️ albumin
- liver failure or nephrotic syndrome
What are some common causes of exudate pleural effusions?
- malignancy
- infection (pneumonia, TB, emphysema)
- autoimmune disease
- chylothorax (blocked lymphatics)
What are some common signs of a pleural effusion in patients?
- ⬇️ chest wall movement on side of effusion
- ⬇️ ventilation on side of effusion
- dullness on percussion (MOST reliable finding)
- ⬇️ tactile vocal fremitus and vocal resonance on side of perfusion
- bronchial breathing above perfusion
- tracheal deviation away from perfusion
In patients with a pneumothorax (collapsed lung), what may they present with?
- sudden shortness of breathe
- sudden chest pain
- BOTH SPONTANEOUS as generally caused by trauma
- WILL BE ON EXAM!!!!!!
Is a pneumothorax (collapsed lung) dangerous?
- yes, can be medical emergency
Why is Boyles Law important in a pneumothorax (collapsed lung)?
- ⬆️ pressure = ⬇️ volume - pressure changes will crush lung cavity
How can a pneumothorax (collapsed lung) be treated?
- lung aspiration using needle - chest drain if required
What may be present on radiography in a patient with a pneumothorax?
- large space
- lung may have collapsed, difference size between lungs
- may cause tracheal deviation
What are some primary risk factors for a pneumothorax (collapsed lung)?
- tall and thin - asthma - collagen vascular disease
What are some secondary risk factors underlying to lung disease for a pneumothorax (collapsed lung)?
- COPD - pulmonary fibrosis - cystic fibrosis
What is the only thing that can cause calcium build up on the walls of the pleural (pleural plaques), not in lungs?
- asbestos inhalation - nothing else can cause pleural plaques WILL BE ON EXAM!!!!!!!
Do chest wall disease affect the lungs ability to perfuse O2?
- no lung tissue is not normally affected
What will a patient with chest wall disease present with in a respiratory clinic?
- progressive worsening of breathlessness
- not sudden though
What are the 3 main groups that can cause chest wall disease in respiratory medicine?
- obesity
- neuromuscular (innervation of muscles of chest wall)
- musculoskeletal
Smoking can cause a combination of obstructive and restrictive lung disease, what is a common example?
- emphysema = OBSTRUCTIVE
- pulmonary fibrosis = RESTRICTIVE
Pulmonary Sarcoidosis can cause a combination of obstructive and restrictive lung disease, what is a common example?
- endobronchial sarcoidosis = OBSTRUCTIVE
- fibrosis = RESTRICTIVE
In an obese patient who smokes this can cause a combination of obstructive and restrictive lung disease, what is a common example?
- smoking = OBSTRUCTIVE - obesity = RESTRICTIVE
Some comon symptoms of a pneumothroax can include breathlessness, chest pain, tachycardia, coughing, tacyopnea and more. Do these symptoms present suddenly or progressively get worse?
- sudden onset of symptoms
- generall caused by trauma
In idiopathic pulmonary fibrosis how long does shortness of breathe need to have been present for?
- >2 years
Why does idiopathic pulmonary fibrosis cause a dry cough?
- no specific known reason
- fibrosis stress on lungs may cause it
- fibrosis may increase sensitivity to coughing triggers
Why does idiopathic pulmonary fibrosis cause a clubbing and hypoxia?
- clubbing = CO2 cannot be sufficiently perfuse out of lungs
- hypoxia = O2 cannot perfuse sufficiently
In idiopathic pulmonary fibrosis is weight loss and fatigue common?
- yes
