Respiratory Flashcards
1
Q
what is chronic obstructive pulmonary disease
A
- chronic lung condition characterised by breathlessness due to poorly reversible and progressive airflow obstruction.
- chronic bronchitis and emphysema
- 1-4 % of population
2
Q
pathology of chronic bronchitis
A
- inflammation and scarring of small bronchioles
- Mucous gland hyperplasia and irritant effects of smoke causes productive cough
3
Q
pathology of emphysema
A
- inflammation and scarring of the small bronchioles
- Imbalance of proteases and antiproteases causes destruction of the lung parenchyma with dilation of terminal airspaces (emphysema) and air trapping
4
Q
pathophysiology of COPD
A
- hyperinflation, thick mucus, dilated terminal airways
- +/- bullae
- finely pigmented macrophages in the respiratory bronchioles
5
Q
clinical manifestations of COPD
A
- exertional breathlessness, history of prolonged cough and sputum
- dyspnoea
- wheeze
- FEV1<80 FEV1/FVC<70
- cyanosis
- cor pulmonale (R heart enlargement)
6
Q
pink puffers
A
- emphysema
- raised alveolar ventilation, a near normal PaO2 and a normal or low PaCO2.
- breathless but are not cyanosed
- may progress to type I respiratory failure
7
Q
blue bloaters
A
- chronic bronchitis
- lowered alveolar ventilation, with a low PaO2 and a high PaCO2.
- cyanosed, may go on to develop cor pulmonale.
- Their respiratory centres are relatively insensitive to CO2 and they rely on hypoxic drive
8
Q
investigations for COPD
A
- FBC = raised packed cell volume (PCV)
- CXR: hyperinflation, flat hemidiaphragms
- CT: bronchial wall thickening, scarring, air space enlargement
- ECG: right hypertrophy (cor pulmonale)
- ABG: low PaO2 with hypercapnia
- Spirometry: obstructive + air trapping
9
Q
management for COPD
A
- exercise, weight loss, stop smoking
- mucolytics (dornase alfa)
- diuretics for oedema
- SABA or SAMA (muscarinic antagonist)
- may need long acting or ICS
10
Q
acute exacerbations of COPD
A
- mostly in winter and triggered by infections
- increased cough, wheeze, breathlessness
- ABG, ECG, FBC, CXR
- ensure oxygenation then treat cause
- salbutamol, IV hydrocortisone, Abx
11
Q
what is asthma
A
- A chronic inflammatory disorder of large airways characterised by recurrent episodes of reversible airway narrowing
- most associated with atopy (asthma, hay fever, eczema) = genetic tendency of immune system to produce IgE in response to common allergens
12
Q
pathology of asthma
A
- produce large amount of allergen specific IgE = binds onto surface of mast cells
- re-exposure = IgE crosslink = degranulation of mast cells
- airway inflammation and bronchospasm
- ongoing inflammation = hypersensitive airways = react to exercise, cold air, cigarette smoke
13
Q
features of asthma
A
- Inflammation of the mucosa
- Increased mucous production
- Bronchodilation
14
Q
clinical manifestations of asthma
A
- Intermittent episodes of breathlessness, wheeze, and chest tightness
- Cough, particularly at night
- NSAIDs and B blockers can precipitate attack
- acid reflux
- poor sleep
- hyperinflated chest
15
Q
investigations for asthma
A
- spirometry, reversibility test (spirometry will increase after dose of salbutamol)
- PEF
- CXR = hyperinflation (chronic asthma)
16
Q
management of asthma
A
- step 1 = occasional SABA
- step 2 = + ICS (beclomethasone 200mcg/day)
- step 3 = Leukotriene receptor antagonist (LRTA, e.g. montelukast)
- 4 = LABA (e.g. salmeterol)
- 5 = Increase ICS dose
17
Q
management of acute severe asthma attack (OSHIT)
A
- OXYGEN to maintain 94-98%
- SALBUTAMOL 5mg nebulised
- add IPRATROPIUM to nebuliser if life-threatening
- iv HYDROCORTISONE/ prednisolone
- THEOPHYLLINE IV (bronchodilator)
18
Q
what is resp failure (type 1 and 2)
A
- Defined as arterial PO2 <8kPa.
- Type 1 = normal or low pCO2
- Type 2 = raised pCO2
19
Q
causes of type 1 resp failure
A
- Severe pneumonia
- Pulmonary embolism
- Acute asthma
- Pulmonary fibrosis
- Acute LVF
20
Q
causes of type 2 resp failure
A
- COPD
- Neuromuscular disorders impairing ventilation e.g. myasthenia gravis
- Reduced respiratory drive e.g. sedative drugs
21
Q
pathology of resp failure (1 and 2)
A
1) increased ventilation removes CO2 but can’t compensate for low pO2 (ventilation/perfusion mismatch)
2) generalised alveolar hypoventilation
22
Q
clinical features of resp failure
A
- hypoxia = dyspnoea, restlessness, agitation, confusion, central cyanosis (long standing hypoxia = pulmonary hypertension and cor pulmonale)
- hypercapnia = Headache, peripheral vasodilation, tachycardia, bounding pulse, tremor/flap, papilledema, confusion, drowsiness, coma
23
Q
investigations for resp failure
A
- Blood tests: FBC, U&E, CRP, ABG
- Radiology: CXR
- Microbiology: sputum and blood cultures
- Spirometry
24
Q
management of type 1 resp failure
A
- Treat underlying cause
- Give oxygen facemask
- Assisted ventilation if PaO2 <8kPa despite 60% O2
25
management of type 2 resp failure
- respiration driven by hypoxia
- treat cause
- controlled O2 therapy (start at 24% O2)
- recheck ABG after 20 min, if PaCO2 lower then increase O2 to 28%
- may need intubation and ventilation
26
what is hypersensitivity pneumonitis/ extrinsic allergic alveolitis
interstitial lung disease caused by an immunologic reaction to inhaled antigens
27
causes of hypersensitivity pneumonitis
- Thermophilic bacteria (mouldy hay, compost, air conditioner ducts)
- Fungi (mouldy maple bark, barley or wood dust)
- Avian proteins (bird droppings and feathers)
28
pathology of hypersensitivity pneumonitis
- Inhaled antigens lead to an abnormal immune reaction in the lungs
- antibody (type 2), immune complex (type 3) and cell-mediated (type 4) hypersensitivity reactions
29
clinical manifestations of hypersensitivity pneumonitis
- acute disease = severe breathlessness, cough and fever 4-6h after large exposure, resolves in 12-18h
- chronic disease = prolonged exposure to small amount of antigen, gradual onset of breathlessness, dry cough, fatigue, clubbing, weight loss, type1 RF
30
investigations for hypersensitivity pneumonitis
- High resolution CT shows middle to upper lobe-predominant linear interstitial opacities and small nodules
- chronic = honey comb lung on CXR, ground glass appearance on CT
31
management of hypersensitivity pneumonitis
- identify cause and avoid
- persistent exposure = fibrosis and honeycomb
- acute =O2, PO prednisolone, reducing course
- chronic = avoid allergen or wear facemask, long term steroids
32
Coal worker pneumoconiosis
- inhalation of coal dust particles over 15-20yrs
= ingested by macrophages which die, releasing enzymes and causing fibrosis
-CXR = round opacities in upper zone
33
progressive massive fibrosis
- due to progression of CWL
- dyspnoea, fibrosis, cor pulmonale
- CXR = fibrotic masses in peripheries of upper-mid zone
34
Caplan's syndrome
The association between rheumatoid arthritis, pneumoconiosis, and pulmonary rheumatoid nodules
35
silicosis
- inhalation of silica particles, very fibrogenic
- metal mining, stone quarrying, pottery
- CXR = nodular pattern, egg-shell calcification of hilar nodes
- increases risk of TB
- spirometry = restrictive
36
asbestosis
- causes pulmonary fibrosis
- dyspnoea, clubbing, fine-end crackles
- increased risk of bronchial adenocarcinoma and mesothelioma
37
byssinosis
- cotton mill worker
- symptoms start on first day back then improve through week
- endotoxins in bacteria in raw cotton
- no CXR changes
38
what is bronchiectasis
- abnormal permanent dilation of bronchi accompanied by inflammation in their walls and in adjacent lung parenchyma
- causes in developed countries = obstruction due to tumour/foreign body or CF related
- post infection (measles, pertussis, bronchiolitis, pneumonia, TB, HIV)
39
pathology of bronchiectasis
- weakening in bronchial walls caused by recurrent inflammation
- Scarring in the adjacent lung parenchyma places traction on the weakened bronchi, causing them to permanently dilate.
- Permanent thinning of these airways.
- Main organisms: H. influenzae, Strep. Pneumoniae, Staph. Aureus, Pseudomonas aeruginosa
40
signs and symptoms of bronchiectasis
- Persistent cough, Copious purulent sputum, Intermittent haemoptysis
- Finger clubbing, Coarse inspiratory crepitations, Wheeze
41
complications of bronchiectasis
- Pulmonary hypertension and RVF
- Pneumothorax
- Deposition of serum amyloid A protein in β-pleated sheets in multiple organs (AA amyloidosis)
42
investigations for bronchiectasis
- Sputum culture
- CXR: cystic shadows, thickened bronchial walls
- spirometry = obstructive
- bronchoscopy
43
management of bronchiectasis
- Airway clearance techniques (chest physio) and mucolytics
- Abx
- bronchodilators
- surgery if severe haemoptysis
44
what is cystic fibrosis
- An inherited disorder caused by a mutation in the cystic fibrosis transmembrane conductance regulator (CFTR) gene
- AR
- CFTR is on chromosome 7q and codes for a chloride ion channel
45
pathology of CF
- deletion of phenylalanine 508 (F508 mutation) causes abnormal folding of the CFTR protein
- Lack of normal CFTR = defective electrolyte transfer across epithelial cell membranes = thick mucus secretions
46
clinical manifestations of CF
- Neonate: failure to thrive, meconium ileus (cause bowel obstruction), rectal prolapse
- Children and young adults: cough, wheeze, recurrent infections, pancreatic insufficiency (DM), gallstones, cirrhosis, male infertility, osteoporosis, arthritis, vasculitis, nasal polyps
- liver failure develops late
47
investigations for CF
- neonatal screening = raised serum immunoreactive trypsin
- sweat test = sodium and chloride >60mmol/L
- CXR = hyperinflation, bronchiectasis
- abdo US = fatty liver, cirrhosis, chronic pancreatitis
- spirometry = obstructive
48
management of CF
- multidisciplinary
- chest physio (postural drainage), bronchodilators
- Abx for infective exacerbations
- treat other symptoms (eg DM), fat-soluble vitamins
- may need ventilation or lung transplant later on
49
what is a pleural effusion
- accumulation of excess fluid within the pleural space
- Blood = haemothorax
- Pus = empyema
- Chyle (lymph with fat) = chylothorax
- Both blood and air = hemopneumothorax
50
what can cause pleural effusion
- VF, Pneumonia, Pulmonary embolism, Malignancy
- Multisystem autoimmune disease e.g. lupus, RA
- Transudates =raised venous pressure (cardiac failure, constrictive pericarditis, fluid overload) or hypoproteinaemia (cirrhosis, nephrotic syndrome, malabsorption).
- Exudates = increased leakiness of pleural capillaries secondary to infection, inflammation or malignancy
51
clinical manifestations of pleural effusion
- small = asymptomatic
- large = breathlessness and pleuritic chest pain
- Decreased expansion, Stony dull percussion note, Diminished breath sounds
52
investigations for pleural effusion
- CXR
- aspiration
- pleural biopsy
53
management of pleural effusion
- drainage
- pleurodesis = irritant drug to stick lung to chest wall
- surgery if persistent and thickening of pleura
54
what is pneumothorax
- air in pleural space
55
causes of pneumothorax
- spontaneous = thin, tall young men (rupture of small apical blebs of lung tissue from stretching of lung)
- underlying lung issue (COPD, asthma, pneumonia)
- Trauma e.g. penetrating chest wound, rib fractures
- Iatrogenic e.g. subclavian vein cannulation, lung biopsy
56
what is a tension pneumothorax
- rare
- tissues near the lung defect and act as a one-way valve, preventing the equalization of pressure = continuous build-up of pressure and volume on each breath
- causes cardiorespiratory arrest
- respiratory distress, tachycardia, hypotension, distended neck veins
57
clinical manifestations of pneumothorax
- Sudden onset of unilateral pleuritic chest pain
- Breathlessness
- asthma/COPD = sudden deterioration
58
investigations for pneumothorax
- CXR will show air (don't do if tension pneumothorax suspected unless air removed first)
- expiratory film
59
management of pneumothorax
- chest drain
| - aspiration
60
what is a lung carcinoma
- malignant epithelial tumour arising in the lung
- small cell carcinoma (SCLC)
- NSCLC = adenocarcinoma, squamous cell carcinoma,
- rare subtypes
61
carcinogenesis of lung carcinomas
- likely to arise from a precursor phase of epithelial dysplasia = neoplastic transformation
- Adenomatous dysplasia/ adenocarcinoma in situ precedes adenocarcinoma
- Squamous dysplasia/ squamous carcinoma in situ precedes squamous cell carcinoma
62
clinical manifestations of lung carcinoma
- local growth = progressive breathlessness, cough, chest pain, hoarseness or loss of voice, haemoptysis, weight loss, and recurrent pneumonia
- metastases = Abdo pain, bony pain, and neuro symptoms
- small cell carcinomas can present with paraneoplastic syndromes (cushings, ADH, PTH, HCG) or the superior vena cava syndrome
63
treatment of NSCLC
- lobectomy
| - radical radiotherapy
64
treatment of SCLC
- surgery
- chemo/radiotherapy
- SVC stent + radiotherapy + dexamethasone
- analgesia, steroids, anti-emetics
65
risk factors of carcinoma of the bronchus
- smoking, asbestos, Chromium, arsenic, iron oxides, and radiation
66
signs of carcinoma of the bronchus
- Cachexia (wasting)
| - Anaemia
67
complications of carcinoma of the bronchus
- Local: recurrent laryngeal nerve palsy
- Phrenic nerve palsy
- SVC obstruction
68
investigations for carcinoma of the bronchus
- CXR: peripheral nodule, hilar enlargement, lung collapse
- Cytology: sputum and pleural fluid
- Fine needle aspiration or biopsy
- Lung function tests
69
bronchial adenoma
- rare, slow-growing. 90% are carcinoid tumours.
| - Treatment – surgery
70
hamartoma
- rare, benign.
- CT: lobulated mass with flecks of calcification
- Excise
71
what is a mesothelioma
- malignant tumour arising in the pleura from mesothelial cells and showing a diffuse pattern of growth over the pleural surfaces
- asbestos fibres become coated in iron (asbestos bodies) and are permanently trapped in lungs
72
clinical manifestations of mesothelioma
- Breathlessness (large pleural effusion)
- Chest pain
- Weight loss and malaise
- Signs of metastases; Lymphadenopathy, Hepatomegaly, Bone pain/tenderness, Abdominal pain/ obstruction
73
management of mesothelioma
- pemetrexed + cisplatin chemo
| - pleurodesis, indwelling pleural drain
74
what is pulmonary hypertension
- mean pulmonary artery pressure >25mmHg at rest or >30mmHg during exercise.
- secondary = complication of chronic lung or cardiac disease
- primary (idiopathic, certain drugs, HIV, collagen vascular disease, congenital systemic-to-pulmonary shunts)
75
pathology of pulmonary hypertension
Chronic hypoxia and obliterative pulmonary fibrosis = raised pressure in the pulmonary arterial circulation
76
clinical manifestations of pulmonary hypertension
- exertional dyspnoea and fatigue
| - dizziness, syncope
77
investigations for pulmonary hypertension
- CXR: enlarged proximal pulmonary arteries which taper distally
- ECG: RVH, and P pulmonale (peaked P waves)
- Echo: right ventricular dilation/ hypertrophy
78
management of pulmonary hypertension
- oxygen
- warfarin (due to high risk of intrapulmonary fibrosis)
- diuretics
- oral CCBs
- treat cause
79
microbiology of TB
- Myocbacterium tuberculosis, an acid-fast rod-shaped bacillus
- Active infection occurs when containment by the immune system (T-cells/macrophages) is inadequate from primary infection or re-activation
- respiratory spread from patient with active pulmonary TB
80
latent TB
- infection without disease
- persistent immune system containment
- Risk factors for reactivation include new infection, HIV, organ transplantation, immunosuppression, silicosis, illicit drug use, malnutrition, high-risk settings
81
pathology of TB
- Inhaled bacilli are engulfed by alveolar macrophages but can survive and multiply within them
- spread to O2 rich sites (lung apices, kidneys, bone, meninges)
- mycobacteria-specific CD4+ helper T-cells are activated following MHC class II antigen presentation
- Th1 subset helper T-cells secrete interferon (IFN)-gamma = macrophages into epithelioid macrophages = granulomas = wall off the mycobacteria in an anoxic and acidic environment = scarring
82
clinical manifestations of TB
- chronic pneumonia = persistent cough, fever, night sweats, weight loss and loss of appetite
- Extrapulmonary TB = Meningitis, Lymphadenopathy, Genitourinary symptoms, Bone or joint pain
- can affect any organ
83
investigations for TB
- acid fast bacilli (Ziehl-Neelsen stain) in sputum, pleural fluid, or bronchoalveolar lavage (BAL) fluid
- culture = definitive but takes 12 weeks
- Tuberculin skin testing (TST) = Mantoux test
Interferon-gamma release assays (IGRAS)
84
treatment for TB
- Rifampicin – 2 months intensive, 4 months continuation
- Isoniazid – 2 months intensive, 4 months continuation
- Pyrazinamide – 2 months intensive
- Ethambutol – 2 months intensive
85
what is pneumonia and its 4 classifications
- infection of the lung parenchyma caused by bacterial organisms
- community-acquired, hospital-acquired, aspiration, immunosuppression
86
microbiology of pneumonia
- Community-acquired: streptococcus pneumoniae, mycoplasma pneumoniae, Haemophilus influenzae, Legionella pneumophila.
- Hospital-acquired: gram-negative bacteria, e.g. Klebsiella, Escherichia coli, Pseudomonas
- Aspiration: mixed aerobic and anaerobic bacteria
- Immunosuppression: all
87
clinical manifestations of pneumonia
- Productive cough
- Breathlessness
- Chest pain
- Fever
- Haemoptysis
- pyrexia, cyanosis, confusion, tachycardia, tachypnoea
88
investigations for pneumonia
- sats, blood tests, sputum culture
- CXR = lobar or multilobar infiltrates, cavitation, or pleural effusion
- check urine for Legionella/ Pneumococcal urinary antigens
89
severity of pneumonia (CURB-65)
- 1 point each
- Confusion
- Urea >7mmol/L
- Respiratory rate >30/min
- BP <90 systolic and/or 60mmHG diastolic
- Age 65+
90
management of pneumonia
- severity 0-1 = Abx/ home treatment
- 2 = hospital therapy
- 3 = severe pneumonia (mortality 15-40%)
- give O2, treat hypotension, keep hydrated, find pathogen, Abx, analgesia
91
what antibiotics for pneumonia
amoxicillin/ flucloxacillin + clarithromycin
92
common cold (acute coryza)
- usually rhinovirus spread by droplets
- incubation 12h-5d
- malaise, slight pyrexia, sore throat, watery nasal discharge
93
sinusitis
- Infection of paranasal sinuses
- Strep pneumoniae or H. influenzae
- frontal headache, facial pain, nasal discharge
- co-amoxiclav, topical corticosteroids ( fluticasone propionate nasal spray), steam inhalations
94
rhinitis
- Sneezing attacks, nasal discharge or blockage occurring for more than 1h for most days;
- For a limited period of the year (seasonal rhinitis) = hayfever = itching of eyes and soft palate
- Throughout the whole year (perennial or persistent rhinitis) = allergic or non-allergic +/- nasal polyps
- skin prick testing
- avoid allergens, antihistamines (loratadine/ cetirizine), beclomethasone spray twice daily (decongestant)
95
acute pharyngitis
- adenoviruses
- sore throat and fever
- More persistent and severe pharyngitis implies bacterial infection – haemolytic Strep, Haemophilus influenzae, staphylococcus aureus = IV penicillin 4x daily for 10d
96
acute laryngotracheobronchitis (croup)
- parainfluenza virus or measles virus
- severe in <3 y/o
- Inflammatory oedema involving larynx = hoarse voice, barking cough (croup) and stridor
- Tracheitis = burning retrosternal pain
- O2, oral/IM corticosteroids, nebulised adrenaline
97
influenza
- mostly type A and B
- coated with haemagglutinin (H) and neuraminidase (N) = for attachment to resp epithelium
- human immunity against surface proteins
- influenza A = antigenic shift = major changes to H and N antigens = pandemic
- seasonal influenza in winter
98
presentation of influenza
Fever, dry cough, sore throat, coryzal symptoms, headache, malaise, myalgia, conjunctivitis, eye pain, photophobia
99
diagnosis of influenza
- clinical = acute onset + cough +fever
- public health surveillance
- viral PCR, rapid antigen testing, viral culture of clinical samples
100
treatment of influenza
- uncomplicated = symptomatic treatment (paracetamol)
- complicated or high risk = antivirals = hospital admission
- antiviral inhibitors of influenza neuraminidase: Oseltamivir, Zanamivir
101
pathology of PE
- thrombus in deep vein (DVT) embolises > R side of heart > pulmonary arterial circulation >loges in pulmonary artery
102
clinical manifestations of PE
- blockage of major artery = instant death due to sudden huge rise in pulmonary arterial pressure
- medium = area of ventilation/perfusion mismatch and breathlessness
- small = subtle breathlessness, chest pain, dizziness
103
investigations for PE
- FBC, U&E, baseline clotting, D-dimers
- ABG may show lowered PaO2 and PaCO2
- Imaging: CXR may be normal, or show oligaemia of affected segment
- ECG: sinus tachycardia
104
management of PE
- O2 if hypoxic
- Aspirin
- morphine IV with anti-emetic
- IV alteplase to thrombolyse massive PE
- if haemodynamically stable then start LMWH
105
prevention of PE
- Give heparin to all immobile patients.
| - Stop HRT and the combined contraceptive pill pre-op
106
what is interstitial lung disease (ILD)
- describe a number of conditions that primarily affect the lung parenchyma in a diffuse manner.
- chronic inflammation and/or progressive interstitial fibrosis
107
clinical features of ILD
- Dyspnoea on exertion
- Non-productive paroxysmal cough
- Abnormal breath sounds
- Abnormal CXR
- Restrictive pulmonary spirometry
108
pathological features of ILD
- Fibrosis and remodelling of the interstitium
- Chronic inflammation
- Hyperplasia of type II epithelial cells or type II pneumocytes
109
classification of ILD
- known cause = occupation, drugs, hypersensitivity etc
- associated with systemic disorders = sarcoidosis, RA, SLE etc
- idiopathic = idiopathic pulmonary fibrosis, non-specific interstitial pneumonitis
110
idiopathic pulmonary fibrosis
- idiopathic interstitial pneumonia limited to the lung
- histological appearance of usual interstitial pneumonia (UIP)
- abnormal repair mechanism to alveolar injury = overexpress profibrotic cytokines = irreversible lung damage
111
clinical manifestations of idiopathic pulmonary fibrosis
- breathlessness, non-productive cough, malaise, weight loss, arthralgia, cyanosis, clubbing, fine end-inspiratory crepitations
112
investigations for idiopathic pulmonary fibrosis
- ABG = low PaO2 (severe = raised PaCO2)
- CXR = decreased TLV, honeycomb lung
- spirometry = restrictive
- lung biopsy
113
management of idiopathic pulmonary fibrosis
- oxygen, pulmonary rehabilitation, opiates, palliative care input
- consider for lung transplant and clinical trials
114
what is sarcoidosis
- multisystem disease of unknown cause in which tissues are infiltrated by granulomas
- granulomatous inflammation
115
clinical manifestations of sarcoidosis
- any organ affected, most common is lymph nodes, lungs and skin
- acute = presents suddenly, erythema nodosum
- chronic = insidious, lupus pernio, pulmonary fibrosis, posterior uveitis
- pulmonary = dry cough, dyspnoea, low exercise tolerance
- lymphadenopathy, hepatomegaly, glaucoma
116
investigations for sarcoidosis
- Blood: raised ESR, lymphopenia
- 24h urine: raised calcium
- CXR is abnormal
- Tissue biopsy: diagnostic and shows non-caseating granulomata
117
management of sarcoidosis
- acute = bed rest, NSAIDs
- corticosteroids if uveitis, hypercalcaemia, neuro/cardio involvement
- severe illness = IV methylprednisolone
118
what is Goodpasture's syndrome ( anti-GBM disease)
- Rare.
- Auto-antibodies to type IV collagen which is present in glomerular and alveolar basement membranes (anti-GBM Abs).
- A pulmonary-renal syndrome
119
clinical manifestations of Goodpasture's syndrome
- Renal disease – oliguria/anuria, haematuria, AKI, renal failure
- Lung disease – pulmonary haemorrhage, SOB, haemoptysis
- crescent glomerulonephritis
120
management of Goodpasture's syndrome
- Plasma exchange
- Corticosteroids
- Cyclophosphamide
- Treat shock
121
what is Wegener's granulomatosis (granulomatosis with polyangiitis [GPA])
- A systemic ANCA-associated vasculitis characterised by dominant upper respiratory tract, lung and renal involvement and cANCA positivity.
- necrotising granulomatous inflammation and vasculitis of small and medium vessels
122
clinical manifestations of Wegener's granulomatosis
- Nasal symptoms – nasal obstruction, ulcers, or destruction of nasal septum
- Acute renal failure
- Pulmonary symptoms – cough, haemoptysis or pleuritis
- Skin purpura or nodules
- Peripheral neuropathy
- Mononeuritis multiplex
123
management of Wegener's granulomatosis
- severe = corticosteroids and cyclophosphamide
| - aggressive immunosuppression needed
