Week Three - Case Two Flashcards
what is the typical presentations of ILD
Often slowly progressive
Occasionally may mimic an acute pneumonia
Dry cough
Progressive shortness of breath
May have clubbing
May have diffuse inspiratory crackles
Wheeze, haemoptysis and chest pain are rare
Investigations
Restrictive respiratory pattern of spirometry
Abnormal CXR
what are the two main types of ILD
pulmonary fibrosis and sarcoidosis
what is pulmonary fibrosis characterised by
scar tissue in the lungs
decreased compliance giving a restrictive pattern in pulmonary function tests (FEV1/FVC >80%)
end stage is honeycomb lung - cystic spaces develop in fibrotic lungs
how can pulmonary fibrosis be classified due to involvement
localised
bilateral
widespread
what are the four main clinical features in fibrotic lung conditions
dry cough
dyspnoea
digital clubbing
diffuse inspiratory crackles
what is idiopathic pulmonary fibrosis / cryptogenic fibrosing alveolitis
relatively rare, progressive, chronic pulmonary fibrosis of unknown aetiology.
occurs in patients 45-65 and involves the lower lobes
what are the main features of idiopathic pulmonary fibrosis / cryptogenic fibrosing alveolitis
breathlessness, dry cough, considerable weight loss and fatigue/malaise.
what are the blood investigations done for ILD - pulmonary fibrosis
FBC (raised ESR), Rh factor (+ve in 50% of patients), ANA (30% +ve)
what does a CXR show for ILD - pulmonary fibrosis
irregular, reticulo-nodular shadows, often in lower zones, sometimes called reticulonodular pattern
CXR is often normal
what will a CT show for ILD - pulmonary fibrosis
Usually a “high resolution CT” (HRCT)
Ground-glass opacification
“Honeycombing”
“Mosaicism”
What kind of pattern is shown on lung function tests - spirometry for ILD - pulmonary fibrosis
restrictive pattern
what will an ABG show for ILD - pulmonary fibrosis
hypoxaemia
what is corticosteroid treatment often combined with
often combined with azathioprine
what is the combined immunosuppressive treatment used for ILDs
Combined immunosuppressive therapy can also be used including azathioprine and cyclophosphamide in conjunction with steroids.
what is the prognosis for idiopathic pulmonary fibrosis
most patients die within 5 years
how is sarcoidosis treated
treatment only indicated if disease is progressive and / or has significant symptoms
Prednisolone – 0.5mg/Kg/day is usually the recommended treatment, for one month, and then weaning to the lowest effective dose, reviewed every 6-12 months
what is coal workers pneumoconiosis
related to total coal dust exposure. it results from inhalation of coal dust over 15-20 years
what are the two syndromes of Coal worker’s Pneumoconiosis
simone pneumoconiosis and progressive massive fibrosis
what is simple pneumoconiosis, what are the symptoms and how is it diagnosed
Simple Pneumoconiosis– Usually asymptomatic, but can have co-existent bronchitis. Diagnosis is made from CXR on finding small, round opacities in the upper zones. Avoidance of exposure to further dust can stop it from progressing. Patients are entitled to claim compensation via the Industrial Injuries Act.
what is Progressive massive fibrosis, what is seen soon chest X rays and what are the symptoms
Large, round fibrotic nodules >10mm seen on CXR, usually upper zones. Nodules can become infected by tuberculosis. The associated emphysema is severe. Cough, dyspnoea and production of black sputum may be present (sputum black due to ruptured, cavitating lesions, hence the name “the black lung”). This can progress despite cessation of exposure to dust, there is no specific treatment. Patients will eventually develop pulmonary hypertension and cor pulmonale.w
what is Caplan’s syndrome
is the associated between CWP (coal workers pneumoconiosis) and rheumatoid arthritis
what are the clinical features of asbestosis
fibrosis, dyspnea, dry cough, clubbing and inspiratory crackles
what kind of cancer is asbestos linked strongly with
mesothelioma
what is extrinsic allergic alveolitis
hypersensitivity pneumonitis and is a widespread inflammatory reaction.
what does extrinsic allergic alveolitis result from
It results from repeated exposure to antigens to which the individual has already been sensitised.
Examples of these antigens include:
Mouldy hay (Farmer’s lung)
Bird Faeces (bird fancier/pigeon fancier’s lung)
Cotton fibres (bysinossis)
Sugar cane fibres (bagassosis)
what are the clinical features of extrinsic allergic alveolitis
Clinical features are SOB, cough, fever which occur hours after antigen exposure. Chronically, features include: weight loss, progressive dyspnoea, type 1 resp. Failure and cor pulmonale.
what is Goodpasture’s syndrome characterised by
glomerulonephritis and respiratory disease together.
what is Goodpasture’s syndrome driven by
It is driven by a type 2 hypersensitivity reaction whereby IgG anti-basement membrane antibodies attach to the glomerulus to cause glomerulonephritis but can also react with the alveolar membrane to cause pulmonary haemorrhage.
The three features to remember are:
Haemoptysis
Haematuria
Anaemia
what is the treatment of Goodpasture’s
corticosteroids, but in some cases plasmapheresis to remove autoantibodies has shown great success
what are the differentials for breathlessness, unremarkable routine blood results and normal echocardiogram?
- Lung cancer
- Interstitial lung disease
- Sarcoidosis
- Asbestos-related lung disease
- Occupational lung disease
- Bronchiectasis
what respiratory conditions are associated with nail clubbing
lung cancer,
suppurative lung diseases (cystic fibrosis, bronchiectasis) and ILD
what can the early findings of ILD be on a CXR
mild basal interstitial changes seen as white lung markings in between the ribs.
this is a very early sign and may not be easily appreciated on chest x-ray imaging
what further investigations should be done to confirm diagnosis of ILD
high resolution CT scan and spirometry
ILD is restrictive, what would the spirometry results look like?
FVC and FEV1 are low, but proportional so, so that the FVC/FEV1 ratio remains normal.
lung volumes are also reduced, as is the transfer factor which measures the lung’s ability to soak up oxygen
what kind of fibrosis is usually seen
it is usually sub pleural, basal and at the end of the lung
what is a definitive diagnosis based on
CT - honeycombing (fibrosis)
what kind of diagnosis is Idiopathic pulmonary fibrosis
diagnosis of exclusion
what crepitations are a feature of fibrotic lung disease
Bi-basal, fine, end-inspiratory crepitations are a pathognomonic feature of fibrotic lung disease. They are sometimes described as ‘velcro-like’
what types of ILD is smoking associated with
Desquamative Interstitial Pneumonia (DIP) and Respiratory Bronchiolitis-Interstitial Lung Disease (RB-ILD) forms of ILD.
what are the ILD conditions with unknown causes
idiopathic pulmonary fibrosis
sarcoidosis
cryptogenic organising pneumonia
what medications are known to cause ILD
methotrexate
Amiodarone
bleomycin
nitrofurantoin
what is methotrexate
an anti-inflammatory known to cause ILD in the form of hypersensitivity pneumonitis
what is Amiodarone
an anti-arrhythmic medication known to cause ILD leading to lung fibrosis
what is bleomycin
a chemotherapy agent
what is nitrofurantoin
an antibiotic known to cause ILD leading to lung fibrosis with lung term use
what are there treatments of idiopathic pulmonary fibrosis
antifibrotics - pirfenidone and nintedanib (slow the progression by about 50%)
pulmonary rehabilitation
oxygen assessment
what are the side effects of antifibrotics - pirfenidone and nintedanib
nausea, reflux, loss of appetite
pirfenidone can cause rashes
what is the immunosuppression treatment for non specific interstitial pneumonia pattern
mycophenolate
prednisolone
