Lung Pathology Flashcards
WHAT ARE THE CAUSES OF OBSTRUCTIVE LUNG DISEASE?
- Asthma
- COPD
- Bronchiectasis
- Bronchiolitis obliterans
What are the causes of restrictive lung diseases?
- Pulmonary fibrosis
- Asbestosis
- Sarcoidosis
- Acute respiratory distress syndrome
- Infant respiratory distress syndrome
- Kyphoscoliosis e.g. ankylosing spondylitis
- Neuromuscular disorders
- Severe obesity
WHAT IS COPD?
Collection of lung diseases that cause IRREVERSIBLE obstruction to airflow out of the lungs.
Umbrella term for two diseases chronic bronchitis and emphysema
What are the causes of COPD?
- Cigarette smoking. (MAINLY)
- Alpha-1-antitrypsin deficiency
- Coal dust exposure
- Cadmium toxicity
What are the non-pharmocological management options of COPD?
- Stop smoking
- Physical activity
-
Vaccinations
- Annaul Flu
- ONE- OFF Pneumococcal
- Pertussis
What is the pharmocological management options of COPD?
- SABA + SAMA is first line
- Asthmatic features?
- Any previous, secure diagnosis of asthma or of atopy
- A higher blood eosinophil count - note that NICE recommend a full blood count for all patients as part of the work-up
- Substantial variation in FEV1 over time (at least 400 ml)
- Substantial diurnal variation in peak expiratory flow (at least 20%)
- No - SABA as required, LABA + LAMA regularly
- Yes - SABA or SAMA as required, LABA + ICS regularly
- SABA as required, LABA + LAMA + ICS
What are the complications of COPD?
- Pneumothorax
- Cor pulmoale
- Acute exaccerbations
- Infection
Under what circumstances is long term oxygen therapy considered in COPD?
COPD - LTOT if pO2 of 7.3 - 8 kPa AND one of the following:
- Secondary polycythaemia
- Peripheral oedema
- Pulmonary hypertension
What are the different stages of COPD?
- Stage 1 (Mild) as his FEV1 is >80% of what is expected.
- Stage 2 (Moderate) is an FEV1 which is 50-79% of the predicted value.
- Stage 3 (Severe) is an FEV1 which is 30-49% of the predicted value.
- Stage 4 (Very severe) is an FEV1 <30% of the predicted value
Post-bronchodilator FEV1/FVC is <0.7
What should be given to COPD patients who have recurrent exaccerbations?
- Home supply of prednisolone and an antibitoic
What are the investigations for COPD?
- Post-bronchodilator spirometry to demonstrate airflow obstruction: FEV1/FVC ratio less than 70%
- Chest x-ray
- Hyperinflation
- Bullae: if large, may sometimes mimic a pneumothorax
- Flat hemidiaphragm
- Also important to exclude lung cancer
- Full blood count: exclude secondary polycythaemia
- Body mass index (BMI) calculation
WHAT IS CHRONIC BRONCHITIS?
https://www.youtube.com/watch?v=Y29bTzKK_P8
Bronchial tubes inflammation
IRREVERSIBLE
What is chronic bronchitis defined as?
Cough, sputum production on most days for 3 months of 2 successive yrs
How can pulmonary hypertension happen in chronic bronchitis?
Decreased gas exchange causign vasocontriction
Large proportion
Increases pulminary vascular resistance
Develops pulmonary hypertension
Right side of heart enlarges
Cor pulmonale
How can a person become cyanosed in chronic bronchitis?
CO2 trapped in alveoli takes up more space
O2 unable to fill alveoli
Less goes into the blood
More CO2 in alveoli means less CO2 can come out the blood
More CO2 in blood less O2
What is the reid index?
Size of the mucinus glands relative to the rest of the layers
Who does chronic bronchitis affect?
Affects middle aged heavy smokers
Some following pollution chronically
Recurrent low grade bronchial infections
What are the symptoms of chronic bronchitis?
- Dyspnea
- Wheeze
- Crackles
- Cough
- Production of mucus (sputum)
What are the signs of chronic bronchitis?
- Hypoxemia
-
Hypercapnia
- These cause cyanosis
- Pulmonary hypertension
-
Result of low O2
- Causing cor pulmonale
- Tachypnoea
- Use of accessory muscles
- Hyperinflation
What are the investigations for chronic bronchitis?
- Spirometry
-
Chest X-ray
- Rule out other lung conditions, such as pneumonia.
-
ECG
- Right venticle and atria enlargment (cor pulmonale)
- Sputum (bacteria).
- ABG: PaO2 decrease ± hypercapnia
WHAT IS ACUTE BRONCHITIS?
Acute bronchitis is a type of chest infection which is usually self-limiting in nature.
It is a result of inflammation of the trachea and major bronchi and is therefore associated with oedematous large airways and the production of sputum
What are the features of acute bronchitis?
- cough: may or may not be productive
- sore throat
- rhinorrhoea
- wheeze
How do you differentitate acute bronchitis from pneumonia?
- History: Sputum, wheeze, breathlessness may be absent in acute bronchitis whereas at least one tends to be present in pneumonia.
- Examination: No other focal chest signs (dullness to percussion, crepitations, bronchial breathing) in acute bronchitis other than wheeze. Moreover, systemic features (malaise, myalgia, and fever) may be absent in acute bronchitis, whereas they tend to be present in pneumonia.
What are the investigations for acute bronchitis?
- acute bronchitis is typically a clinical diagnosis
- however, if CRP testing is available this may be used to guide whether antibiotic therapy is indicated
What is the management for acute bronchitis?
- analgesia
- good fluid intake
- consider antibiotic therapy if patients:
- are systemically very unwell
- have pre-existing co-morbidities
- have a CRP of 20-100mg/L (offer delayed prescription) or a CRP >100mg/L (offer antibiotics immediately)
- NICE Clinical Knowledge Summaries/BNF currently recommend doxycycline first-line
- doxycycline cannot be used in children or pregnant women
- alternatives include amoxicillin
WHAT IS EMPHYSEMA?
https://www.youtube.com/watch?v=TEuSV_7gWA8
Enlargement of alveolar airspaces with destruction of elastin in walls.
What happens when the alveoli becomes irritated by smoke?
What does this do?
What happens when exhaling?
- Triggers an inflammatory response, immune cells come in
- Releasing inflammatory mediators (Proteases, Collangenases)
- Breaks down the collagen and elastin in the alveoli wall
Bernoulli principle
- Exhalation of air has a lower pressure and pulls the airway inwards, ormal airways can withstand this
- Emphysema airways have less elastin and collagen therefore collapse
Leads to air trapping
What happens when air comes into emphysema lungs?
Lungs are more compliant
Lungs overinflate
What happens in alpha-1 antitrypsin deficiency?
- Macrophages produce proteases
- Helps clear debris
- Can damage tissue
- Alpha-1 antitrypsin is a protease inhibitor
- Protects against damage
- Deficiency means proteases can damage lungs
What are the oxygen and carbon dioxide levels in emphysema?
‘pure’ emphysema appears with reduced PaCO2 and normal PaO2 at rest due to overventillation (‘pink puffers’)
What are the symptoms of emphysema?
Dyspnea - shortness of breath
Exhaling through pursed lips to keep lungs inflated
Cough - sputum
Weight loss due to metabolic demands
1/3 of lung capacity is destroyed before symptoms
What are the signs of emphysema?
- Barrel chest - overinflated chest on x-ray
- Flattened diaphragm
- Pulmonary hypertension
- Right heart failure
What are the investigations for emphysema?
-
Pulmonary/Lung Function tests
- Spirometry is the best test to detect airflow limitation and obstruction.
-
Blood tests
- Hb level increase secondary polycythaemia.
-
Chest x-ray
- This helps to show hyperexpansion of the lungs.
-
ECG or Echocardiogram
- Right heart failure (a complication of emphysema and COPD).
-
Blood gases
- Normal, later stages of disease, low oxygen and high carbon dioxide levels.
-
High resolution CT
- Best test for detecting emphysema and bullae (big dilated air spaces).
WHAT IS AN EXACERBATION OF COPD DEFINED AS?
An exacerbation can be defined as a sustained worsening of the patient’s symptoms from his or her usual stable state that is beyond normal day-to-day variations, is acute in onset and requires additional therapy
What are the most likely causative organism in an exacerbation of COPD?
- H. influenzae - MOST COMMON
- S. pneumoniae
- Moraxella cartarrhalis
What are the clinical features of an exacerbation of COPD?
- worsening of previous stable condition
- increased wheeze
- increased dyspnoea
- increased sputum volume
- increased sputum purulence
- chest tightness
- fluid retention
- reduced exercise tolerance
- increased fatigue
What are the investigations for an exacerbation of COPD?
- Many clinical diagnosis
- Sputum samples
- Pulse oximetry is of value if there are clinical features of a severe exacerbation
- X-ray
- ABG
- ECG
- FBC
What is the treatment for an exacerbation of COPD?
- Increase Bronchodilator use and consider giving via a Nebuliser
- Give Prednisolone 30 mg daily for 5 days
- Oral antibiotics IF PURULENT SPUTUM
- First-line: Amoxicillin or clarithromycin or doxycycline
When should NIV be considered for acute exaccerbation of COPD?
- Respiratory acidosis
- PaCO2 > 6kPa
- pH <7.35 ≥7.26
- Persists despite immediate maximum standard medical treatment
What is given for prophylaxis against exaccerbations in COPD?
Azithromycin 250mg 3 times a week
WHAT IS BRONCHIECTASIS?
https://www.youtube.com/watch?v=rTcVPHszU5E
Irreversible dilation of the bronchioles
What are the causes of bronchiectasis?
-
Congenital
- Cystic fibrosis (CF);
-
Post-infection
- TB
- HIV
-
Immune deficiency
- Selevtive IgA, hypogammaglobulinaemia
-
Other
- Bronchial obstruction (tumour, foreign body)
What is the pathology of bronchiectasis?
Infection and imparied drainage
Activates neutrophilic proteases and cytokines causing inflammation
Increaed mucus and increased stagnent bacteria
Cycle starts again
What are the signs and symptoms of bronchiectasis?
- Chronic cough - foul smelling sputum
- Flecked with blood sometimes.
- Finger clubbing
- Coarse inspiratory crepitations.
- Wheeze
What are the tests for bronchiectasis?
- Sputum culture
-
Chest X-Ray
- Cystic shadows, thickened bronchial walls
-
HRCT chest
- To assess extent and distribution of disease.
-
Spirometry
- Obstructive
What is the management for bronchiectasis?
- Stop smoking
- Airway clearance exercises
- Bronchodilators
- Corticosteroids
-
Antibiotics - if infective cause
- Amoxicillin
-
Surgery in selected cases
- Disease confined to one lobe
What are the complications with bronchiectasis?
Pneumonia
Emphysema
Septicaemia
Amyloid formation.
WHAT IS ALPHA-1 ANTITRYPSIN DEFICIENCY?
A deficiency in alpha-1 antitrypsin
What does alpha-1 antitryspin do?
What is the gene that codes for it?
What chromosome is it on?
Inactivates elastase (a protease)
SERPINA1
Long arm of chromosome 14
When would you favour alpha 1-antitryspin deficiency as a dignosis?
- Never smoked
- Young
- Lower lobes affected
What are the symptoms of alpha-1 antitrypsin deficiency?
- Shortness of breath
- Wheezing
- Mucus production
- Chronic cough
Cirrhosis
- Inability to make coagulation factors
- Build up of toxins
What investigations are done for alpha-1 antitrypsin deficiency?
How do you diagnose a child in utero with this?
-
Bloods
- A1AT low
-
Biopsy
- Liver - Periodic-acid shiff stain
-
Spirometry
- Obstructive picture
-
Diagnose a child
- Amniocentesis
What is the treatment of alpha-1 antitrypsin deficiency?
- No smoking
-
Supportive
- Bronchodilators, physiotherapy
- Intravenous alpha1-antitrypsin protein concentrates
-
Surgery
- Lung volume reduction surgery
- Lung transplantation
WHAT ARE INTERSTITIAL LUNG DISEASES?
Disease of the alveoli primarily
Scarring
Fibrosis
What are some causes of interstitial lung disease?
Congenital
Neurofibromatosis
Tuberous sclerosis
Chemical or dust inhalation
Organic dusts e.g. moulds, avian proteins
Mineral dusts e.g. silica, coal dust, asbestos
Unknown causes:
Idiopathic haemosiderosis
Sarcoidosis
What are the symptoms of interstitial lung diseases?
- Dry cough
- Digital clubing
- Diffuse inspiratory crackles
- Dysponea
What can acute interstitial lung diseases cause?
Acute respiratory distress syndrome
WHAT ARE SOME CAUSES OF ACUTE RESPIRATORY DISTRESS?
- Pancreatitis
- Shock
- Trauma
- Infections
- Gas inhalation
- Narcotic abuse
What are the symptoms of acute respiratory distress syndrome?
- Tachypnoea
- Dyspnoea
- Pulmonary oedema
WHAT IS PNEUMOCONIOSIS?
Lung disease caused by inhaled dust Organic or inorganic dust (mineral)
What happens in coal workers’ pneumoconiosis?
Coal is ingested by alveolar macrophages (dust cells)
They aggregate around bronchioles
The consequences vary from trivial to lethal.
WHAT IS RHEUMATOID ARTHRITIS INTERSTITIAL LUNG DISEASE?
Rheumatoid arthritis interstitial lung disease (RA-ILD) is increasingly recognised and is clinically significant in up to 5% of patients with RA
What are the investigations for rheumaotid arthritis interstitial lung disease?
- Pulmonary function testing
- High resolution CT
What is the treatment for rheumatoid arthritis interstitial lung disease?
High-dose corticosteroids
Prednisolone
Immunosuppresants
Azathioprine
What is Caplan’s syndrome?
The association between rheumatoid arthritis, pneumoconiosis, and pulmonary rheumatoid nodules.
How do you treat coal workers pneumoconiosis?
Avoid exposure to coal dust
Treat co-existing chronic bronchitis
WHAT IS ASBESTOSIS?
Pneumoconiosis caused by inhalation of asbestos fibres, classically causing a chronic, diffuse, interstitial fibrosis of the lun
What is asbestosis caused by?
Inhaltion of asbestos.
What are the features of asbestosis?
Similar to other fibrotic lung diseases with progressive dyspnoea, clubbing, and fine end-inspiratory crackles.
Also causes pleural plaques, increased risk of bronchial adenocarcinoma and mesothelioma.
What are the investigations for asbestosis?
Chest X-ray
Diffuse bilateral shadowing, honeycomb lung. There may be pleural plaques which are an indicator of previous exposure to asbestos
Lung function tests
Restrictive
What is the management of asbestosis?
Symptomatic.
Patients are often eligible for compensation through the UK Industrial Injuries Act.
WHAT IS EXTRINSIC ALLERGIC ALVEOLITIS?
https://www.youtube.com/watch?v=PIuj6XiMlps
Inflammation of the alveoli due to an extrinsic allergen of known type
What are the differnt types of extrinsic allergic alveolitis?
-
Bird fanciers’ lung
- Avian proteins from bird droppings
-
Farmers lung
- Spores of Saccharopolyspora rectivirgula from wet hay (formerly Micropolyspora faeni)
-
Malt workers’ lung
- Aspergillus clavatus
-
Mushroom workers’ lung
- Thermophilic actinomycetes*
What is the pathology of EAA?
What type of sensitivity is it?
Allergens comes in
Irritates alveoli and activates neutrophils
Releases inflammatory cytokines
Cascade leads to B cell antibody prodction
Complexes form
TYPE 3
What are the two durations of EEA?
Acute (few hours after only inflammation)
Chronic (fibrosis)
What is seen in chronic that is not seen in acute EAA?
Granulomas
Non-caseating
Macrophages fuse with eachother making granulomas
What are the symptoms of extrinsic allergic alveolitis?
-
Acute (occurs 4-8 hrs after exposure)
- Dyspnoea
- Dry cough
- Fever
-
Chronic (occurs weeks-months after exposure)
- Lethargy
- Dyspnoea
- Productive cough
- Anorexia and weight loss
What are the tests for extrinsic allergic alveolitis?
-
CXR
- Upper-zone mottling/consolidation
- Honeycombing sometimes
-
Blood
- FBC (neutrophilia) but NO esosinophilia
- ESR increase; ABGS; positive serum precipitins (indicate exposure only).
-
Lung function tests
- Restrictive defect; reduced gas transfer during acute attacks.
What are the management options for extrinsic alergic alveolitis?
- Remove allergen
- O2
- Oral prednisolone
What is the difference between asthma and EAA?
Asthma
Obstructive
Larger airways
Type 1 allergic
EAA
Restrictive
Smaller airways
Type 3 and 4 allergic
WHAT IS SARCOIDOSIS?
https://www.youtube.com/watch?v=D-ahTqbqnqE
Granulomatous disease affecting mainly the lungs, but also lymph nodes in a greater frequency.
What type of granuloma is sarcoidosis?
Non-ceasiating granulomatous disease
What is the epidemology of sarcoidosis?
- African-americans
- Women more than men
- Under 40 years of age
- Non smokers
What is the cause of sarcoidsis?
What cells are disregulated?
Unknown
Disregulation of CD4 cells
What are the pulmonary symptoms of sarcoidosis?
4 D’s!
Dry cough
Digital clubbing
Dyspnoea
Diffuse inspiatory crackles
What are the non-pulmonary symptoms of sarcoidosis?
- Skin – erythema nodosum
- Arthritis - esp. of feet, hands
- Cardiac– heart block, VT, heart failure
- Ocular– anterior uveitis, can lead to blindness
- Intracranial (brain) – chronic meningitis, seizures, neuropathy
- Derangement of liver and renal function – hepatic granuloma (70% patients), hypercalcaemia(
What are the tests for sarcoidosis?
-
Tissue biopsy
- Diagnostic non-caseating granulomatas
-
X-ray
- Enlarged lymph nodes
-
24h urine
- Ca2+ increase.
-
Blood
- Increased ESR, lymphopenia, LFT incerease,
- Serum ACE increase
- Increased Ca2+
What is the management of sarcoidosis?
- Nothing usually
-
Indications for steroids
- Patients with chest x-ray stage 2 or 3 disease who are symptomatic. Patients with asymptomatic and stable stage 2 or 3 disease who have only mildly abnormal lung function do not require treatment
- Hypercalcaemia
- Eye, heart or neuro involvement
What does facial rash (lupus pernio) plus lymphadenopathy indicate?
Sarcoidosis
What is an acute version of sarcoidosis called?
Lofgren sydrome
WHAT IS IDIOPATHIC PULMONARY FIBROSIS?
Uknown excess amount of collagen in the lungs connective tissue and interstitial tissue after damage
What is the cause of IPF?
Uknown
What are the risk factors for IPF?
- Old age
- Smoking
- Male
What happens with fluid in IPF?
Loss of alveoli and start pooling fluid
Seen as honeycombing
What are the symptoms of IPF?
4 D’s!
Dry cough
Digital clubbing
Dyspnoea
Diffuse inspiatory crackles
What are the tests for IPF?
-
CXR
- Thickening of alveoli walls
- Honeycomb lung
-
CT
- Needed for diagnosis
-
Spirometry
- Restrictive FEV1 LOW, FVC LOW
- TLCO REDUCED
- Decrease transfer factor
-
Blood
- ABG decrease in oxygen, increase in CO2
- CRP increase; immunoglobulins increase;
- ANA (30% +ve), rheumatoid factor (10% +ve).
- BAL (Bronchoalveolar lavage)
What are the management options for idiopathic pulmonary fibrosis?
- Pulmonary rehabilitation
- Pirfenidone (an antifibrotic agent) may be useful in selected patients (see NICE guidelines)
- Many patients will require supplementary oxygen and eventually a lung transplant
WHAT ARE THE TWO TYPES OF LUNG TUMOUR?
- Bronchial
- Pleural
What are the different types of bronchial cancers?
Malignant (95%) = lung cancer
- Non small cell cancer
- Small cell cancer
Benign
- Hamartoma
- Carcinoid
- Lipoma
What are the causes of lung cancer?
- Smoking
- Asbestos
- Chromium
- Arsenic
What are the cell types of lung cancer?
- Small Cell Lung cancer - 15%
-
Non Small Cell lung cancer - 85%
- Adenocarcinoma (adenocarcinoma-in situe) - MOST COMMON
- Squamous
- Large cell 5%
- Alveolor Cell Carcinoma
- Bronchial adenoma - mostly carcinoid
What are the symptoms of local lung cancer?
Cough (40%)
Chest pain (20%)
Haemoptysis (7%)
What are the signs of lung cancer?
Weight loss
Anaemia
Clubbing
Supraclavicular or axillary nodes
What are the features of lung adenocarcinomas?
- Most common in non smokers
- Gynaecomastia
- Hypertrophic pulmonary osteoarthropathy (HPOA)
What are the features of small cell lung cancer?
- MOST COMMON IN SMOKERS
- Usually central
- Arise from APUD* cells
- Associated with ectopic ADH, ACTH secretion
- ADH → hyponatraemia
- ACTH → Cushing’s syndrome
- ACTH secretion can cause bilateral adrenal hyperplasia, the high levels of cortisol can lead to hypokalaemic alkalosis
- Lambert-Eaton syndrome: antibodies to voltage gated calcium channels causing myasthenic like syndrome