resp middle tier Flashcards
idiopathic pulmonary fibrosis = in what group of diseases? age? gender? cause?
most common interstitial lung disease
60y+
male 2x more likely
idiopathic (perhaps repetitive alveolar epithelium damage in response to unknown environmental stimuli causes uncontrolled repair of damaged tissue)
risk factors for idiopathic pulmonary fibrosis
Triggers of incorrect wound healing (Excess fibrosis)
- Cigarette smoking
- infection -(CMV EBV Hep C)
- Occupational exposure - dust, metals, woods
- Drugs - methotrexate, impiramine (antidepressant)
- GORD
- Genetic predisposition
idiopathic pulmonary fibrosis pathophysiology
- where
- pattern of fibrosis
- inflammation?
- Progressive, chronic fibrosis of lung interstitium (distal to terminal bronchiole→ alveolar, capillary interface),
- causing restrictive respiratory defect
- Patchy fibrosis
- Minimal /absent inflammation
- Acute fibroblastic proliferation -overproduction of fibroblasts
- Collagen deposition
- Causes structural integrity of lung parenchyma to be disrupted
- Loss of elasticity
- Ability to perform gas exchange impaired (progressive respiratory failure)
idiopathic pulmonary fibrosis signs
Clubbing
Inspiratory basal crackles
Cyanosis
idiopathic pulmonary fibrosis symptoms
Breathlessness -exertional
Non-productive cough (→ respiratory failure, pulmonary hypertension, cor pulmonale)
Weight loss
Malaise
Arthralgia
idiopathic pulmonary fibrosis investigations and what is found
CXR/CT (CT more sensitive)
- honeycomb lung (ground glass)
- reticular shadowing at peripheries
- more basally
- bronchiestasis
bloods
- ABG - hypoxia, co2 normal (high if severe)
spirometry
- restrictive =
FEV1/FVC = above 70%
FVC less than 80% predicted value
possible lung biopsy
idiopathic pulmonary fibrosis management
- Lung testing to monitor disease progression
- Supportive
- – Oxygen inc portable (ambulatory)
- – Pulmonary rehab
- – Palliative care - opiates
- Treat other
- – GORD
- – Cough
- Antibiotic pirfenidone – slows rate of FVC decline
- NO prednisolone (think- theres not inflammation anyway)
- Lung transplant (only years left after this)
- Low survival - 2-5years
idiopathic pulmonary fibrosis complications
respiratory failure pulmonary hypertension cor pulmonale PE Pneumothorax Resulting infection
sarcoidosis complications
renal damage
respiratory failure
sarcoidosis treatment
treat only if :
developed, symptomatic, pulmonary infiltration (substance denser than air – pus, blood, protein. seen on x-ray)
- prednisolone (methotrexate if resistant)
otherwise:
bed rest, NSAIDs, extrapulmonary features/complications addresssed
sarcoidosis investigations
CXR
- bilateral hilar lymphadenopathy = enlarged, white bits either side of the mediastinum
- pulmonary infiltration (Substance denser than air - pus, blood, protein.)
Bloods
- high Ca
- high ESR
- high ACE
- low lymphocytes (lymphopenia)
- high immunoglobulins
- raised LFTs
broncho-alveolar lavage
- shows increased lymphocytes in active disease
- increased neutrophil if fibrosis present
tissue biopsy
- diagnostic. shows ** non-caseating granulomas **
x-ray hands/ feet
- may show punched-out lytic lesions in terminal phalanges
ECG
- may show arrythmias / bundle branch blocks
sarcoidosis presentation
50% asymptomatic - often detected on routine x ray
Lung :
- Dyspnoea - progressive. Reduced exercise tolerance
- Non-productive cough
- Chest pain
Skin :
- Erythema nodosum
- Lupus pernio (Red/purple) - Often over cheeks/nose/ears
Eye :
- Granulomatous uveitis
- Conjunctivitis
- Glaucoma
- Fever
- Weight loss
- Fatigue
- Hepatomegaly
- Lymphadenopathy (enlarged)
Other
- Bell’s palsy - facial nerve lesion
- Facial numbness
- Dysphagia
- visual field defects
- Renal stones – Hypercalciuria
- Cardiac arrhythmias
- Heart block
sarcoidosis pathophysiology
inc
- acute/chronic
- where
- what is formed
a multisystem chronic inflammatory condition
formation of non-caseating epithelioid granulomata (macrophages, lymphocytes - T cells, epithelioid cells)
extracellular matrix deposition
type of interstitial lung disease
thoracic cavity - mediastinal lymph nodes -- bilateral, hilar - pulmonary infiltrations skin eyes
sarcoidosis
- age
- race
- risk factors
- cause
20-40y more severe in A-C highest prevalence in n europe risk factors = family history, genes, race genetics - HLA DRB1, HLA DQB1 unknown cause
HLA DRB1
HLA DQB1
sarcoidosis
pneumonia =
inflammation of lung usually due to infection
pneumothorax =
collapsed lung, due to leaked air between lung and chest wall which compresses it in
give three categories of bronchiectasis causes + examples
+ two extras
post- infection (most common)
- pneumonia
- measles
- whooping cough
- TB
- pertussis
- bronchiolitis
congenital
- CF
- deficiency of bronchial wall elements
- primary ciliary dyskinesia (kartageners syndrome)
mechanical bronchial wall obstruction
- foreign body
- post TB stenosis
- lymph node, tumour
+ COPD, asthma complication
+ immunodeficiency
main infective organisms - bronchietasis
Haemophilus influenzae Strep pneumonia Pseudomonas aeruiginosa Moraxella catarrhalis Staph aureus
bronchietasis epidemiology
- gender
- age
elderly women
how do you differentiate TB from bronchiectasis
sputum culture
bronchiectasis pathophysiology
- Irreversible abnormal dilatation of bronchi and bronchioles with chronic inflammation
- Scarring /fibrosis makes them thickened due to infection / obstruction
- Airway distortion and dilation
- Mucus builds up – Permanent dilation means muco-ciliary clearance impaired
- Cilia lost
- Bronchial tree colonised
- Exacerbations occur: Secondary Infections chance increased – recurrent (muco-ciliary clearnance impaired). This leads to further distruction
- Usually lower lobes affected
bronchiectasis presentation
Chronic cough Copious sputum production Purulent (pus, foul smelling) - suggests infection Discolured - khaki Chest pain Dyspnoea , SOB Haemoptysis - intermittent Coarse crackles (inspiratory) Wheeze Clubbing - nail, finger General ill health exarcerbations + long recovery time
bronchiectasis investigations
sputum culture
Xray / CT (HRCT better)
- bronchial wall dilation – signet ring appearance (dilation is the ring bit, pulmonary arteries are the signet bit)
- Thickening
also
- spirometry – obstruction
- sweat test - exclude CF
- bronchoscopy - exclude foreign bodies and look for site of hemoptysis
- blood : looking for inflammatory marker
CF complications
Male infertility
Pancreatitis
Recurrent respiratory tract infections
Bronchiettasis
CF surgical treatment
bilateral lung transplant
CF pharmacological treatment
- Bronchodilators
- Prophylactic antibiotics
- Anti-mutolytics
- Enzymes (for pancrease) inc insulin
- Bisphosophonates - bone strength improvement (less nutrients absorbed)
- Vaccines for infections eg flu
- Vitamin supplemets (ADEK)
- PPI - to reduce stomach acidity
non pharmacological CF treatment
- Chest physio - airway clearance (postural drainage), airway techniques
- High protein intake
- Genetic counselling
- Monitor lung function
- Stop smoking
- Screening for osteoperosis
CF investigations
Sweat test
- Measure salt in sweat
- Diagnostic . specific
Genetic testing (UK babies screened at birth)
- Look for gene mutation
- Diagnostic
Faecal elastase in newborns = marker or pancreatic damage . low elastase in CF patients .
also
- Bloods
- Bacterial culture
- Radiology
- Abdominal ultrasound
- Spirometry : obstruction — monitor
presentation of CF
- when
childhood
clubbing salty sweat wheeze rectal prolapse failure to thrive- low weight recurrent infections cough heavy mucus production (thick, purulent) nasal polyps crackles streatorrhea respiratory obstruction hemoptysis
CF pathophysiology
- general
- mutated CFTR gene (chloride transport on epithelial surfaces)
- Cl- (and bicarbonate) not secreted into lumen
- Na enters cell to balance
- water enters /doesnt leave the cell
- thick, dehydrated secretions/ lumen
how does CF affect
- bones
osteoperosis : bone strength reduced as. less nutrients absorbed
how does CF affect
- gonads
male infertility (Atrophy of vas deferns + epididymus)
amennorhea
how does CF affect GI / pancreas / gall bladder
- pancreatic insufficiency = pancreatic secretions are dehydrated –> enzyme stagnation –> secretions blocked due to viscous mucus (diabetes, streatorrhea, maldigestion, malabsorption)
bile more concentrated –> damage
Gi secretions are low volume and viscous and water deficient (intestinal obstruction, reduced GI motility)
how does CF affect airways
Airway dehydration → decreased mucociliary clearance (mucus stasis) and bacterial colonisation → inflammatory lung damage due to neutrophil response
- Originates in small airways, leading to progressive obstruction : Viscid (viscous) secretion plug off small airways → bronchiectasis
- Airways chronically colonised by bacteria
- – neonates/infants : staph aureus
- – infants/children: haemophilius influnzae
- – Older children/adults: pseudomonas (mucoid/non-mucoid)
- Chronic infection → progressive airflow obstruction, cor pulmonale, death
CF cause
race
Genetically inherited - autosomal recessive
- CFTR gene on chromosome 7
- Codes for CFTR - cystuc fibrosis transmembrane regulator protein
- Most common mutation is DF508
caucasian, less common in A-C, asian
what treatment might you consider for a patient with recurrent pleural effusions
Pleurodesis with talc
Irritable substance instigates inflammatory reaction → visceral and parietal adhere so no space for effusion
(recurrence often in malignancy)
what can you do with a pleural fluid sample
Appearnace
- Purulent in empyema
- Turbid (cloudy/opaque) in infected effusion
- Milky in chylothorax (lymph)
pH – Acidic indicates infection
Cytology – looks for wbc and malignant cells
Microbiology – looks for infective organisms
= determine cause
how much fluid is needed to see pleural effusion on chest x ray
300-600ml
normally contains roughly 30ml
what might you hear on chest examination in pleural effusion
what might you see
- Stony dull percussion ipsilateral
- Diminished breath sounds ipsilateral
- Decreased tactile vocal fremitus (this is the vibration felt on chest/back when words spoken. Decreased because transmission of sound reduced in liquid of effusion)
- less vocal resonance
- reduced chest expansion ipsilateral
pleural effusions =
- categorised
Excessive accumulation of fluid in pleural space
- Fluids (effusions) with low concentration of protein (<25g/L) = transudates (transparent- less protein)
- Fluids (effusions) with high concentration of protein (>35g/L) = exudates (exudes protein so cloudy)
possible causes of pleuritic chest pain
pneumothorax PE pleural effusion trauma cancer autoimmune - SLE viral infection
treatment for Plural effusion
Drain effusion
- If exudate and if symptomatic
Pleurodesis with talc
- Irritable substance instigates inflammatory reaction → visceral and parietal adhere so no space for effusion
prevents reoccurance (malignancy)
Treat underlying cause
Surgery - for persistent collections / increasing pleural thickness
pleural effusion symptoms
May be asymptomatic Dyspnoea - esp on exertion, SOB Pleuritic chest pain Cough Weight loss (malignancy)
chylothorax=
pleural effusion with lymph
empyema =
pleural effusion with pus
haemothorax =
plerual effusion with blood
causes of transudate pleural effusion
LOW PROTEIN
Decrease in fluid resporption
- Hypoproteinemia (malabsorption, cirrhosis, nephrotic syndrome) - Not enough protein in blood to draw water back so it remains
Increase in fluid production
- Increased venous pressure (cardiac failure, pericarditis, fluid overload)
?
Peritoneal dialysis (uses peritoneum as filter)
Hypothyroidism
causes of exudate pleural effusion
HIGH PROTEIN Leaky capillaries in pleura (damaged pleura) due to infection, inflammation, malignancy Pneumonia Malignancy Lymphoma Mesothelioma TB Pulmonary infarction - PE Asbestos pancreatitis MI Is deposited in nearby tissues
What non-pharmacological measure would you recommended to prevent recurrent episodes?
stop smoking
What are the boundaries of the triangle of safety with regard to a chest drain? (used in pneumothorax)
- Lateral edge of pec major
- Base of axilla
- Lateral edge of latissimus dorsi
- 5th intercostal space
A tall thin man comes into A&E with severe shortness of breath and pleuritic chest pain. His trachea is deviated from the midline to the right side of his body. One side of his chest is hyperresonant and has reduced expansion. He is a smoker. What does this patient have and which side of the body is affected?
what should the next step be
tension pneumothorax. L side
immediate needle thoracostomy/aspiration + oxygen - skip investigations, this is too much of an emergency and cardiorespiratory arrest may occur if carry out x ray and air isnt removed immediately
pneumothorax complications
recurrence is common
cardiac arrest
death
pneumothorax treatment
- Stop smoking (prevent recurrence)
- Oxygen mask
- Aspiration - remove air, decollapse lung
- Intercostal drain (when aspiration fails / trauma = cause / haemothoraax / mechanical ventilation . Done in triangle below armpit)
Recurrent pneumothorax
- pleurodesis with talc
- Stop smoking
- Surgery
Tension – skip investigations, immediate action needed
- Aspiration
- Oxygen
- Treat underlying cause eg wound
- Small spontaneous ones can heal on their own – air reabsorbed
investigations for pneumothorax
CXR
- Black = air. Water appears white
- Helps for seeing size of small pneumothorax
- Checks if aspiration has been successful
ABG - check for hypoxia in dyspnoaeic patients
Not for tension – this is too much of an emergency and cardiorespiratory arrest may occur if delay and air isnt removed immediately
symptoms of pneumothorax
May be no symtpoms- esp if young, fit Sudden onset Breathless , dyspnoea Pleuritic chest pain - sharp May be worse on inhalation
signs of pneumothorax
- reduced chest expansion ipsilateral
- absent breath sounds ipsilateral
- low O2
- low BP
- pallor
- tachypnoea
- tachycardia
- hyperresonant percussion ipsilateral
tension pneumothorax additional signs
trachea deviated away from pneumothorax
Severe respiratory distress
Shock
Cardiorespiratory arrest
pneumothorax =
types of pneumothorax
Collection of air in pleural cavity → pressure pushes in on lung + elastic recoil → lung collapse/ deflation
Simple
- There is a two wave valve in the pleura (air escapes from here)
- Closed = pleural cavity pressure < atmospheric pressure
- Open = pleural cavity pressure = atmospheric pressure
- Trachea usually central
Tension
- Pleural tear acts as a one way valve – each breath, more air is dragged into the pleural space but no air is allowed out of the cavity
- Unilateral pleural cavity pressure increases
- Pleural cavity pressure > atmospheric pressure
- Trahcea usually deviated away from pneumothorax
Pneumothorax leads to significant impairment of respiration and/ or blood circulation
pleural cavity / airway communication
open/closed
If the communication open… = bronchopleural fistula
If the communication closes… air is reabsorbed into lung over time and reverts to normal
- Small spontaneous ones can heal on their own
causes/ risk factors for pneumothorax
- Spontaneous (Talll thin male.. m>f)
- Chronic lung disease (asthma, COPD, CF, lung fibrosis, sarcoidosis) - rupture of subpleural bullae (fluid filled bilster sacs)
- Infection (TB, pneumonia, lung abscess)
- Trauma (inc iatrogenic eg aspiration)
- Carcinoma
- Smoking
- Mechanical ventilator
- Connective tissue disease (Marfan’s syndrome, Ehler’s Danlos syndrome)
- Family history
Primary - congenital , trauma, spontaneous in healthy people
Secondary - underlying disease
pulmonary hypertension causes
increase in pulmonary vascular resistance or increase in pulmonary blood flow
- PE /veno-occulsive disease
- primary pulmonary hypertension
- sickle cell
- portal hypertension
- chronic lung disease eg COPD
- cardiac disease eg LV HF, tumour, congential, mitral stenosis
- SLE
also
-HIV, morbid obesity, MG, and more
pulmonary hypertension definition
increase in mean pulmonary arterial pressure (mPAP) – above 25 mmHg
narrower vessels
pulmonary hypertension effects
- RV pressure increase as a result → RV must pump harder → RV hypertrophy and dilatation –> cor pulmonale (R sided enlargement and dilation of pulm vessels too)
- Pulmonary endothelium damaged → vasoconstictors released eg endothelin (worsens)
presentation of pulmonary hypertension
Exertional
- Dyspnoea
- Lethargy
- Fatigue
- Due to inability to increase cardiac output with exercise
Abdominal pain (from hepatic congestion)
Oedema (due to RV failure)
also:
- chest pain, syncope, loud pulmonary sound
pulmonary hypertension investigations
CXR
- Enlarged proximal pulmonary arteries
- enlarged heart
- may reveal cause
ECG
- RV hypertrophy
also:
- LFTs, echocardiogram, autoimmune screening
pulmonary hypertension treatment
Treat underlying cause Oxygen Warfarin (intrapulmonary thrombosis) Diuretics (oedema) CCB to vasodialte
heart transplant if young
endothelin receptor antagonist
