The respiratory system - Breathlessness: restrictive lung disease Flashcards
Name the 5 R’s of inflammation and repair
- Recognition - of the injurious agent
- Recruitment - of the leukocytes
- Removal - of the agent
- Regulation - of the response
- Resolution/repair
What is acute respiratory distress syndrome (ARDS)?
ARDS is defined as non-cardiogenic pulmonary oedema and diffuse lung inflammation, typically secondary to an underlying illness.
Describe the pathophysiology of ARDS
ARDS involves a diffuse bilateral alveolar injury, as a result of inflammation mediated by inflammatory mediators such as TNF-α, IL-1 and IL-8.
The resulting endothelial injury activates neutrophils in the pulmonary capillaries, releasing reactive oxygen species and proteases that damage the alveolar endothelium and type 2 alveolar cells.
As a result, the vascular permeability increases and the lung surfactant is lost.
Fluid accumulation in the alveoli causes pulmonary oedema and subsequently hypoxaemia.
Causes of acute respiratory distress syndrome
a) Direct lung injury
b) Indirect lung injury
a)
Common causes
- Pneumonia
- Aspiration of gastric contents
Uncommon causes
- Pulmonary confusion
- Near drowning
- Inhalation injury
- Fat emboli
- Repercussion injury
b)
Common causes
- Sepsis
- Severe trauma with shock
- multiple transfusion
Uncommon causes
- Post cardiac surgery
- Pancreatitis
- Drug overdose
- After massive transfusion
Clinical features of ARDS
a) Symptoms
b) Signs
a) Acute onset respiratory failure which fails to improve with supplemental oxygen, the symptoms of which include severe dyspnoea, tachypnoea, confusion, and presyncope
b) Fine bibasal crackles, but no other features of heart failure
CXR changes in ARDS
Bilateral alveolar infiltrates, without other features of heart failure (such as cardiomegaly and Kerley B lines).
What is the histological pattern of acute respiratory distress syndrome
Diffuse alveolar damage
Management of ARDS
- Ventilatory support: a low tidal volume is associated with better outcomes.
- Haemodynamic support to maintain mean arterial pressure >60 mmHg.
- DVT prophylaxis.
- Nutritional support with enteral/parenteral means if necessary.
- Regular repositioning of patient for pressure ulcer prophylaxis.
- Antibiotics need only be administered if an infectious cause for the ARDS is identified (such as pneumonia or sepsis).
What is Type 1 respiratory failure?
Hyperaemia (PaO2 < 8 kPa) and a normal or low CO2
What is type 2 respiratory failure?
Hyperaemia (paO2 < 8 kPa) and hypercapnia (PaCO2 > 6.5 kPa)
Name 5 causes of T1RF
Ventilation-perfusion mismatch
Decreased atmospheric pressure
Shunt
Pneumonia
ARDS
Pulmonary embolism
Why does T2RF occur?
The rise in PaCO2 is no longer matched by an increase in alveolar ventilation. This can be because:
- Ventilatory drive is insufficient
- The work of breathing is excessive
- The lungs are unable to pump air in and out
As a result, patients are unable to ‘blow off’ the excess CO2 causing PaCO2 levels to rise
a) What can acute T2RF lead to?
b) 3 common causes of acute T2RF
a) Respiratory acidosis (as the excess CO2 is converted to carbonic acid in the blood stream)
b)
Exacerbations of COPD, severe asthma, CF and bronchiectasis
Respiratory depressants (e.g., opiate overdose)
a) How is chronic T2RF evidenced on ABGs
a) How is chronic T2RF evidenced on ABGs
b) 4 common causes
a) Normal pH, elevated PaCO2 and elevated bicarbonate levels
b)
- COPD
- Asthma
- Chronic neurological disorders (e.g.m motor neurone disease)
- Chronic neuromuscular disorder (e.g., myopathies)
- Chest wall diseases
- Obesity hypoventilation syndrome
Clinical features of hypoxia (T1RFF and T2RF)
- Dyspnoea
- Restlessness and agitation
- Confusion
- Cyanosis
Clinical features of hypercapnia (T2RF only)
- Headaches
- Drowsiness
- Confusion
- Tachycardia with a bounding pulse
- CO2 retention hand flap
- Peripheral vasodilation
- Papilloedema
Describe the investigations for respiratory failure
Definitive diagnosis – ABG sampling
Oxygen saturation monitoring
Further investigations to find underlying cause:
- Blood tests and blood cultures
- Chest radiograph
- Sputum culture
- Bedside spirometry testing
- ECG: look for cardiac arrhythmias secondary to hypoxaemia and acidosis
What is the defenitive diagnostic investigation for respiratory failure?
ABG sampling
Describe the management for respiratory failure
- Treat the underlying cause
- Adequate oxygenation and respiratory support (type of support required depends on the type and severity of respiratory failure):
- Oxygen delivery: via nasal canula, a face mask, a venturi mask, or a non-rebreather mask
- Non-invasive ventilation: e.g., continuous Positive Airway Pressure (CPAP) ventilation or a Bilevel Positive Airway Pressure (BiPAP) ventilation
- Invasive ventilation: via an endotracheal tube or a tracheostomy
a) Why must you take special care in oxygenation in patients at risk of T2RF e.g., severe COPD patients
b) What is the target
Respiratory drive in these patients has become relatively insensitive to high PaCO2 and so their drive to breathe is stimulated by low PaO2 (hypoxic drive to breath).
Over oxygenation leads to suppression of ventilation and PaCO2 may rise rather than fall (oxygen-induced hypercapnia)
This can result in respiratory acidosis which can be fatal if not recognized and treated aka decompensated T2RF
Name 3 estrictive extra-pulmonary disease affecting:
a) neuromuscular
b) Chest wall
a)
- Diaphragmatic paralysis
- Cervical spine injury
- Myasthenia Gravis
- Guillain-Barre
- muscular dystrophies
b)
- Kyphoscoliosis
- Obesity
- Ankylosing spondylitis
What is interstitial lung disease?
Refers to a family of conditions with shared characteristics of interstitial inflammation, fibrosis and/or cellular changes in the absence of infection of malignancy
ILD can be divided into main classifications. What are they?
- Known cause association
- Idiopathic interstitial pneumonias (IIP)
- Granulomatous
- Other ILDs (cystic)
Provide the subtypes of ILD due to known cause association
- Drug side-effects
- Connective tissue disease
- Occupational ILD
Provide the subtypes of ILD due to idiopathic interstitial pneumonias
Idiopathic pulmonary fibrosis
Other IIPs
- Respiratory bronchiolitis (RBILD)
- Non-specific pneumonia (NSIP)
- Desquamative interstitial pneumonia (DIP)
- Lymphocytic intersiital pneumonia (LIP)
- Cryptogenic organising pneumonia (COP)
- Acute intersitital pneumonia
Provide the subtypes of granulomatous ILD
- Sarcoidosis
- Hypersensitivity pneumonitis (Extrinsic allergic alveolitis)
Provide the subtype of other ILDs (cystic)
Lymphangio-leimyomatosis (LAM)
Langerhans cell histiocytosis X (LCH)
What is the most common form of idiopathic interstitial pneumonia
Idiopathic pulmonary fibrosis
Clinical features of idiopathic pulmonary fibrosis
a) Symptoms
b) Signs
a)
- Exertion dyspnoea
- Dry cough
- Fatigue
- Arthralgia
b)
- Fine bi-basal end inspiratory crackles
- Clubbing
- Reduced chest expansion
- Acrocyanosis ( bluish discolouration of the extremities due to decreased amount of oxygen delivered to the peripheral part)
Describe the conservative, medical and surgical management of IPF
Conservative
- supportive care
- Smoking cessation
- Pulmonary rehabilitation
Medical
- Oxygen therapy: LTOT and if symptoms persist at rest, low dose opiates or benzodiazepines may be commenced
- DMARDS: Pirfenidone and nintedanib
Surgical
- lung transplant
Complications of IPF
- T2RF
- Increased risk of lung cancer
- Cor pulmonale
Prognosis of IPF
Disease progression and prognosis varies greatly between individuals and is difficult to predict.
There is a median survival of 3 years after diagnosis
Only 2 DMARDs are linseed to treat IPF. What are they?
Pirfenidone
Nintedanib
Pirfenidone
a) MOA
b) Side-effetcs
a) anti-inflammatory and anti-fibrotic
b) Nausea and photosensitivity rash
Nintedanib
a) MOA
b) Side-effetcs
a) Acts on 3 growth receptor factors implicated in the development of iPF
b) Nausea
Describe the CXR changes in IPF
Bilateral lower zone reticule-nodular shadowing may be seen
Describe the HRCT changes in IPF
Honeycombing
Reticular opacities
Traction bronchiectasis
Emphysema
Loss of lung volume
Non-specific interstitial pneumonia (NSIP)
a) Epidemiology
b) What may it be associated with
c) HRCT changes
a) 40-50 years, M=F
b) may be associated with CTDs
c) Ground-glass, reticular shadowing
Respiratory bronchiolitis ILD (RBILD)
a) Why does it occur?
b) HRCT changes
a) Exaggerated bronchiolitis response to smoking
b) patchy ground glass, centrilobular micro nodules, regional attenuation
Desquamative intersiitital penumonia
a) Who does it occur in?
b) HRCT changes
a) Heavy smokers
b) Ground glass opacities, bronchial thickening
Hypersensitivity pneumonitits
a) What is it?
b) Breath sounds on examination
c) What is seen on biopsy?
d) Treatment
a) Type III and type IV hypersensitivity reaction to antigens, causing inflammation of the alveoli, bronchioles, peri-bronchioles
b) Crackles, wheeze, squeaks
c) Granulomas in alveolar space and bronchioles
d) Removal froma antigen, steroids
Give the causes of hypersensitivity pneumonitis and also the corresponding disease name
Actinomycetes in hay and or straw - farmers lung
Avian proteins fro feathers and droppings - bird fanciers lung
Mouldy barley - malt workers lung
Aspergillus in mushroom compost - mushroom workers lung
Actinomycetes n water reservoirs (air conditioning units) - ventilation pneumonitis
Lymphagioleimyomatosis (LAM)
a) What is its?
b) Epidemiology
c) What does it also cause
d) Treatment
a) Abnormal growth of smooth muscle cells, especially in the lungs and lymphatic system
b) Females, mid 30s
c) chylothorax (lymph formed in the digestive system (chyle) accumulates in your chest cavity)
Angiomyolipomas (benign tumors formation in kidneys)
d) Sirolimus (aka rapamycin is a immunosuppressant)
Langerhans cells histiocytosis
a) What is it?
b) Epidemiology
c) Treatment
a) A multi-system disorder in which excess immune system cells called Langerhans cells build up in the body.
b) Young smokers, (20-40yrs), M:F
b) Smoking cessation, steroids
Langerhans cells histiocytosis
a) What is it?
b) Epidemiology
c) Treatment
a) A multi-system disorder in which excess immune system cells called Langerhans cells build up in the body.
b) Young smokers, (20-40yrs), M:F
b) Smoking cessation, steroids
Describe the investigations in idiopathic pulmonary fibrosis
Bloods - FBC, Renal function, lots, cap
ABG
Lung function tests - spirometry, diffusion capacity of the lung for carbon monoxide (DLCO)
Imaging
- CXR
- High-resolution CT
Biopsy and cytology
- Bronchoalveolar lavage and/or transbronchial biopsy
- Surgical lung biopsy
a) Describe the spirometry changes in ILDs e.g., IPF
b) Which IPF shows a variable pattern in spirometry
a) Associated with a restrictive pattern
FEV1 and FVC both reduced
FEV1/FVC > 0.7
b) Sarcoidosis
How does ILDs e.g, IIPF affect the diffusing capacity of the lung for carbon monoxide decreased (DLCO)
Decreases
Describe the HRCT changes in ILD
- Ground glass
- Consolidation
- Non-specific
- Airspace shadow
- Fibrosis: Reticular shadowing, traction bronchiectasis, honeycombing
Describe the conservative and pharmacological treatment of ILD
Conservative
- Education
- Patient support group
- Pulmonary rehabilitation
- Palliative care
Pharmacological
1. Fibrosis - anti-fibrotic for IPF
2. Inflammation - corticosteroids, methotrexate/hydoxychlroroquine, azathioprine/mycophenolate,cyclophosphamide
Pulmonary fibrosis
a) Signs
b) Causes
a) Fine end-inspiratory crepitations
b)
Lung damage: Infarction, pneumonia, tuberculosis
Irritants: Coal dust, silica
Diffuse parenchymal lung disease: Idiopathic pulmonary fibrosis and extrinsic allergic alveolitis
Connective tissue disease: RhA, SLE Systemic Sclerosis and Sjögren’s syndrome
Medications: Amiodarone, Nitrofurantoin, Bleomycin, Methotrexate
Hypersensitivity pneumonitis: Exposure to birds or moulds
Describe the differential diagnosis for chronic dyspnoea
Respiratory
- Airways: asthma, COPD
- Parenchymal: ILD
- Pleura and chest wall
- Pericardium: effusion/constriction
Cardiac
- Muscle: ischaemic heart diseases, cardiomyopathies
-Valve: stenosis, regurgitation
- Rhythm: tachyarrythmia, bradycardia
- Pericardium: effusion/constriction
Other
- Thyrotoxicosis
- Anaemia/haemorrhage
- Obesity
- Anxiety
What is sarcoidosis?
Sarcoidosis is a rare multi system granulomatous disorder of Unkown aetiology
Describe the epidemiology of sarcoidosis
- More common in women with a 2:1 female-male-ratio
- Most often diagnosed between 25-40 years
Describe the pathophysiology of sarcoidosis
Requires two things
- Genetically primed individual
- Exposure to susceptible antigen
Antigen and host proteins from a poorly soluble compound
APCs activate macrophages and dendritic cells producing cytokines
T-helper type 1 promoted by cytokines help form epithelium granulomas
A combination of genetic predisposition and environmental can predispose patient sot sarcoidosis. Give an example for the following:
a) Genetic predispositions
b) Environmental exposures
a)
- Multiple areas of the HLA region
- BTNL2
- ANXA11
b)
- Beryllium, silica, other dusts
- Mycobacterium tuberculosis and Propionibacterium catalases
- Moulds
Describe the presentation of acute sarcoidosis
Flu-like illness
Lofgren’s syndrome (Bilateral hilar lymphadenopathy, erythema nodosum, arthralgia)
Give the different manifestations of sarcoidosis
- Pulmonary
- Ocular
- Cutaneous
- Constitutional
- Musculoskeletal
- Abdominal
- Gi and genito-urinary
- Cardiac
- Hereford’s syndrome (aka uveoparotid fever)
- Lofgren’s syndrome (acute sarcoidosis)
Describe the symptoms of different manifestations of sarcoidosis
Ocular
- Eye pain
- Visual disturbance
- Vision loss
Pulmonary
- Breathlessness (exertion)
- Cough
- Wheeze
Skin
- Rashes (erythema nodosum, subcutaneous nodules, lupus pernio)
Joints
- Joint pain and swelling
- Joint stiffness
- Skin rashes
- Myalgia and weakness
Neurological
- Seizures
- Headaches
- Cranial nerve palsies
Cardiac
- Arrhythmias
- Ventricular failure
- Sudden cardiac death
GI/urogenital
- Abdominal pain
- Haematuria
- Deranged LFTs
- Change in bowel habit
- Infertility
Incidental Hypercalcaemia
Constitutional
- Fatigue
Name the top 3 manifestations in sarcoidosis
- Pulmonary (90%)
- Ocular (30-60%)
- Cutaneous (25%)
Pulmonary sarcoidosis
a) Manifestations
b) Symptoms
c) Signs
d) Complications
a)
- Lymphadenopathy (mediastinal and hilar): bilateral and symmetrical
- Parenchymal disease: ILD
- Airway disease: asthma-like symptoms/bronchial hyper-reactivity, airway stenosis
b) Progressive breathlessness
c)
- Fine inspiratory crackles
- Exertional desaturations
- Clubbing
d) Pulmonary artery hypertension, cor pulmonale
Ocular sarcoidosis manifestations
- Uveitis
- Keratoconjunctivitis sicca
- Adnexal granuloma
- Secondary glaucoma
Cutaneous sarcoidosis manifestations
Papular sarcoidosis: multiple papules develop, generally on the head and head areas
Erythema nodosum
Lupus pernio: a violaceous, nodular rash distributed over the nose and cheeks
Describe the presentation (briefly) of chronic sarcoidosis
Progressive symptoms - e.g., dyspnoea, cough
Associated extra-pulmonary disease and organ dysfunction
Describe the different manifestations of sarcoidosis
Constitutional - fatigue, weight loss, arthralgia and low grade fever. Lymphadenopathy and enlarged parotid glans
Calcium metabolism - Hypercalcaemia/vit D deficiency
Pulmonary -Llymphadenopathy, ILD, airway obstruction and stenosis
Ocular - uveitis, keratoconjuctivitis sicca, adnexal granuloma, secondary glaucoma
Cutaneous - papular sarcoidosis, erythema nodosum, lupus pernio
Musculoskeletal - bone cysts, arthralgia, arthritis and enthesitis
Renal disease - occurs secondary to hypercalcaemia which ay cause nephrocalcinosis
GI and genitourinary
Abdominal - hepatomegaly, splenomegaly, renal stones, IBD mimic, infertility
Neurological - mononeuropathy/polyneuropathy, meningitis, raised intracranial pressure, psychiatric problems
Cardiac - arrhythmias, cardiomyopathy and HF
Hereford’s syndrome (aka uveoparotid fever) - characterised by uveitis, parotid swelling, fever, and facial nerve palsy
Lofgren’s syndrome (acute sarcoidosis) - bilateral hilarity lymphadenopathy, erythema nodosum, arthralgia and fever
What is Heerfordt’s syndrome (aka uveuoparotid fever) characterised by?
Uveitis, parotid swelling, fever, and facial nerve palsy
What is Lofgren’s syndrome characterised by?
Bilateral hilar lymphadenopathy, erythema nodosum, arthralgia and fever.
What important extra-pulmonary manifeststations require treatment?
Cardiac
- Dyspnoea
- Collapse
- Cardiomyopathy
- Sudden cardiac death/arrhythmias
- Pulmonary hypertension
Calcium metabolism
- Hypercalcaemia
- Hypercalciuria/renal calculi
Ophthalmic
- Uveitis
- Visual loss
Neurological
- Seizures
- Aseptic meningitis
- Cranial nerve palsies
Give the investigations for sarcoidosis and there indications
Bedside
- Observations
- Mantoux test: To distinguish from TB, as TB and sarcoidosis cause cavitating lesions
Bloods
- FBC: lymphopenia, anaemia
- Renal function: assess for renal disease
- LFTs: assess for liver disease
- ESR/CRP
- Bone profile: assess for hypercalcaemia
- Serum ACE: raised in around 70%. However, it is NOT specific
- Immunoglobulins: associated with Ig deficiencies (particular IgA)
ECG
- assess for arrhythmias
Imaging
- CXR
- HRCT
Special
- Spirometry
- Tissue biopsy: to confirm diagnosis
- Lumbar puncture: if CNS disease suspected
Describe the CXR changes in sarcoidosis
- Bilateral hilar and mediastinal lymphadenopathy
- Reticulonodular opacites
- Airspace opacities
- Pulmonary fibrosis
Describe the HRCT changes in sarcoidosis
- Lymphadenopathy
- Diffuse nodularity
- Ground glass opacification
- Fibrosis (upper lobe predominance)
Scaffold staging may be used to stage pulmonary sarcoidosis based upon chest radiograph findings.
Describe the chest radiograph findings for the following stages.
a) Stage 0
b) Stage I
c) Stage II
d) Stage III
e) Stage Iv
a) Normal
b) Bilateral hilar lymphadenopathy (BHL)
c) BHL with pulmonary infiltrates/parenchymal changes
d) pulmonary infiltrates/parenchymal changes without BHL
e) Advance pulmonary fibrosis
a) What investigation confirms the absolute diagnosis of sarcoidosis
b) Is it always required?
a) Tissue biopsy
b) No
In what instant may a tissue biopsy for sarcoidosis not be required?
If Lofgren’s syndrome or chronic stable CT changes typical of sarcoidosis and after monitoring patient is well
What type of lung biopsy is best for predominantly nodal disease (Stage I/II) sarcoidosis
Endobronchial ultrasound-transbronchial needle aspiration (EBUS-TBNA)
+/- bronchoalveolar lavage (BAL)
What type of lung biopsy is best for predominantly parenchymal disease sarcoidosis
Transbronchial biopsy
+/- bronchoalveolar lavage (BAL)
What type of lung biopsy is best for mixed nodal and parenchymal sarcoidosis
Endobronchial ultrasound - Transbronchial (EBUS-TBNA)
+/- Bronchoalveolar lavage (BAL)
Name the classic histopathological finding in sarcoidosis
Non-caseating granuloma
Describe the conservative management of sarcoidosis
- Offered support and counselling
- Optimisation of lifestyle factors e.g., smoking cessation, weight loss, dietary advice
Give the indications stated by the British Thoracic Society of when treatment should be started if sarcoidosis
Potential danger of a fatal outcome or permanent disability
OR
Unacceptable loss of QoL
Describe the 1st, 2nd and 3rd line pharmacological management of pulmonary disease in sarcoidosis
1st line - steroids
- High dose, typically 20-40mg daily for 4-6 weeks which is tapered down over 6-18 months until a maintenance dose, typically 5-10mg a day is reached
2nd line - Classical immunosuppressants e.g., Methotrexate 10-15mg (+ Folic acid) (best evidenced 2nd line), Azathioprine, Hydroxychloroquine, Leflunomide and Mycophenolate mofetil
3rd line - Biologics e.g., anti-TNF such as infliximab
What are the indications for second line (immunosuppressant therapy) treatment of pulmonary sarcoidosis
- Significant side-effects from steroids
- Co-morbidities putting patients at greater risk of steroid-related side effects
Progressive pulmonary disease / unacceptable ongoing symptoms
Inability to taper steroids to an acceptable maintenance dose
Patient aversion to steroid therapy - may be used as initial therapy
Describe the surgical options for pulmonary sarcoidosis and the indications
Lung transplant - indicated in those with:
- Advanced pulmonary fibrosis
- Pulmonary hypertension
Describe the prognosis of sarcoidosis
Acute and incidental findings have better outcomes
Severe manifestations are associated with higher mortality
Name the 5 most common occupational respiratory diseases
- Occupational asthma
- COPD
- Asbestos related
- Silicosis
- Pneumoconiosis
- Extrinsic Allergic Alveolitis (EAA)
Name 5 asbestos related diseases
- Pleural plaques
- Diffuse pleural thickening
- Malignant mesothelioma
- Asbestosis
- Lung cancer
- Laryngeal cancer
a) What is asbestos?
b) Describe how the different size and length of asbestos play a role in asbestosis
a) Asbestos is an industrial term for naturally occurring fibre silicates
b)
- Particles < 10µm may penetrate alveoli
- Long straight fibres can reach the alveoli despite their length being > than 10µm
- Fibres > 5 µm more resistant to clearance by phagocytosis
a) What is asbestosis and what is characterised by?
b) When do symptoms of asbestosis develop?
a) Asbestosis is a form of pneumoconiosis caused by inhalation of asbestos fibres, which is characterised by scarring and inflammation
b) Symptoms typically start to develop decades following exposure to asbestos
Clinical features of asbestosis
- Dyspnoea
- Non-productive cough
- Weight loss
- Fine inspiratory crackles
- Finger clubbing
- Susceptibility and deterioration associated with smoking
Describe the investigations for asbestosis
- Spirometry
- Transfer factor
- CXR
- HRCT
- Lung biopsy
Describe the findings for following investigations in asbestosis
a) Spirometry
b) Transfer factor
c) CXR
d) HRCT
e) Lung biopsy
a) Usually a restrictive pattern
b) Reduced
c) Fine nodular shadowing
d) Fibrosis
e) Interstitial fibrosis and asbestos bodies
Describe the management of asbestosis
- Supportive
- Smoking cessation
Mesothelioma
a) What does it have a strong association with?
b) When do symptoms develop?
c) Symptoms
d) Name 3 industries that increase risk of mesothelioma
e) Prognosis
a) Asbestos exposure
b) > 20 years after asbestos exposure
c) Chest pain, SOB
d)
- Plumbers/ electricians/ maintenance
- Demolition
- Construction
- Plumbing and pipe lagging
- Braking lining manufacture/mechanics
- Railway engineering
- Asbestos mining
If occupational asthma is diagnoses, who should it be reported by and to who?
If diagnosed it should be reported by the employer to HSE under RIDDOR (Reporting of injuries, Diseases and Dangerous Occurrences Regulations)
Presentation of occupational asthma
Initially symptoms of wheee, dyspnoea, and chest tightest at work or after work, improving over week
Overtime this pattern can be lost
List 5 occupations and their causes that may lead to occupation asthma
Paint spraying - Isocyanates
Cleaners - cleaning agents
Bakers - flour
Laboratory workers - animals
Electronics - rosin flux fume
Healthcare - latex
Carpentry - wood dust
Detergent manufacture - enzymes
Hairdressers - persulfate salts
What serial PEFR is diagnostic of occupational asthma?
Serial peak flow of > 20% variation across shift and lower/more variable peak flow on workdays
Describe the management of occupational asthma
- Management is that of the BTS guidelines for asthma
- Elimination of exposure (symptoms usually resolve after this)
What is pneumoconiosis
A group of diseases caused by inhalation of mineral dust
Give 5 pneumoconiosis and the mineral dust that causes it
Coal workers pneumoconiosis - coal dust
Asbestosis - asbestose
Silicosis - silica
Slate works pneumoconiosis - slate
Kaolin - china clay
Stannosis - tine ore
Siderosis - iron oxide
Simple coal worker pneumoconiosis
a) symptoms
b) Spirometry changes
c) CXR changes
a) Often symptomatic
b) Can show obstructive (emphysema) or restrictive (fibrosis) pattern
c) Nodular opacities
Progressive massive fibrosis
a) What is it?
b) Symptoms
a) Fibrotic masses, in upper or middle zones, 3-10cm diameter and can cavitate
b) Dyspnoea and productive cough (black sputum)
Acute silicosis (silicoproteinosis)
a) Symptoms
b) CXR changes
c) Treatment
a) Progressive dyspnoea and cough
b) Pulmonary oedema
c) No specific treatment
Name 3 causes of occupational COPD
Mineral dusts e.g., coal, silica, cement
Organic dusts e.g., wood, flour, cotton (Byssinosis)
Chemical e.g., isocyanates, cadmium, welding fumes
Name 3 occupational respiratory infections
Viral respiratory tract infections
Tuberculosis
Legionnaires disease
Psittacosis (Chlamydia psticcaci)
Name 3 occupational causes of lung cancer
- Passive smoking
- Mineral dusts
- Asbestos
- Arsenic
- Nickel
Name the 4 common (histological) subtypes of rheumatoid arthritis - ILD
- Idiopathic/usual interstitial pneumonia (UIP)
- Nonspecific interstitial pneumonia (NSIP)
- Lymphocytic intersiital pneumonia (LIP)
- Organising pneumonia (COP)
Name the 4 common (histological) subtypes of RA - ILD
- Idiopathic/usual interstitial pneumonia (UIP)
- Nonspecific interstitial pneumonia (NSIP)
- Lymphocytic intersiital pneumonia (LIP)
- Organising pneumonia (COP)
Which lobe is affected in RA-ILD?
Lower lobe
What is there treatment for RA-ILD
- Methotrexate
- Anti-TNF alpha
- Corticosteroids (best for with with organising pneumonia subtype)
Which type of systemic sclerosis is ILD most commonly associated with?
Diffuse cutaneous SSc
Which antibody is strongly associated with ILD in systemic sclerosis
Scl-70
ILD in systemic sclerosis
a) Symptoms
b) Management
a) Breathlessness and cough
b)
- Cyclophosmade (most effective)
Various immunnossuppressive agents used: Steroids
- Colchicin
- D-penicillamine
Chloambucil
ILD in systemic sclerosis
a) Symptoms
b) Management
a) Breathlessness and cough
b)
- Cyclophosmade (most effective)
Various immunosuppressive agents used: Steroids, colchicine, D-penicillamine, Chlorambucil
Lupus pneumonitis (ILD - SLE)
a) Signs on histology
b) What must you exclude in the ddx and why?
c) Treatment
a) Feature of interstitial pneumonitis
b) Important to exclude infections as they might present in similar fashion as acute lupus pneumonitis
c) Heavy immunosuppression and plasmapheresis (Corticosteroids + Azathioprine or cyclophosphamide)
Name 3 ways that ILD in dermatomyositis may present
- Rapidly progressive Acute interstitial pneumonia/Hamman-Rich syndrome
- Slowly progressing symptoms of usual interstitial pneumonia ) or cryptogenic organising pneumonia
- Abnormal CXR/HRCT/PFT but no respiratory symptoms
ILD - Sjrogen’s syndrome
a) Respiratory symptoms
b) Treatment
a) Cough and breathlessness
b)
Immunosuppressants: corticosteroids and azathioprine/cyclophosphamide
ILD - mixed connective tissue disease
a) What is the ILD spectrum of this similar to?
b) In which patients is the degree of lung fibrosis more severe in?
c) Treatment
d) In what patients is treatment poor in?
a) ILD spectrum similar to that seen in SSc
b) The degree of lung fibrosis is severe in patients with SSc features
c) Corticosteroids and cyclophosphamide
d) Response not very good in patients with SSc like disease
Methotrexate lung injury (methotrexate interstitial pneumonitis)
a) Symptoms
b) Pulmonary function test findings
c) CXR changes
d) HRCT changes
e) Treatment
a) cough, dyspnoea, fever
b) restrictive defect, low diffusion capacity
c) alveolar infiltrates, reticulonodular shadowing, predominantly diffuse or lower lobe involvement
d) Patchy ground glass shadowing
e) Discontinuation of therapy alone, corticosteroids
Is there a correlation between ILD progression and control of connective tissue disease?
No
Name the roles involved in the MDT care of ILD
- ILD consultant
- ILD CNS
- Radiologist
- Thoracics
- Pathologists
- Pharmacists
- Dieticians
- Physiotherapists
- OTs
Name the roles involved in the MDT care of ILD
- ILD consultant
- ILD CNS
- Radiologist
- Thoracics
- Pathologists
- Pharmacists
- Dieticians
- Physiotherapists
- OTs
- Pulmonary rehab
