Interstitial lung disease Flashcards
What is ILD?
lung disease associated with interstitium , tissue around the alveoli
what is the most common ILD type?
idiopathic pulmonary fibrosis
others: systemic sclerosis, Lupus
what is idiopathic pulmonary fibrosis?
progressive scarring and fibrosis of the lung interstitium
no discernible underlying cause
what drugs (name 3 ) that can cause IPF?
methotrexate, amiodarone, bleomycin
IPF is common over what age group?
> 60 yrs
Is there ethnic factors involves in IPF?
no, but a familial variant (FPF, Familial Pulmonary Fibrosis) exists
What are the clinical features of IPF?
seen at an older age Progressive dyspnoea worse on exertion cough finger clubing (may be seen) >bilateral inspiratory crackles< restrictive pattern on spirometry
Investigation of IPF
examination and history Peak flow test DLco (reduced) CXR (bilateral lower zone reticulonodular shadows) HRCT= round glass appearance
Reticulation (net like) results from thickening of the interlobular or intralobular septa and appears as several linear opacities that resemble a mesh or a net on HRCT scans.
Reticular= too many lines
nodular=too many dots
management of IPF (idiopathic pulmonary fibrosis)?
pulmonary rehabilitation
supportive care
guidelines recommend the use tyrosine kinase inhibitotrs or pirfenidone
average life expectancy is 3-5 years from the time of diagnosis
What is sarcoidosis
chronic granulomatous disorder of unknown cause
multi-organ system disorder
histologically=non-caseating granulomas
Who are the most commonly affected by sarcoidosis?
Higher incidence in Afro-Caribbean and
Scandinavian populations
Traditionally affects people in their second to
fourth decade
Clinical features of sarcoidosis
Flu like illness-pyrexial
dry cough
cutaneous changes=erythema nodosum
maculopapular=both flat and raised rash
Anterior uveitis (inflammation of the middle layer), dry eyes
Hypercalcaemia (from granulomatous change in the disease, due to macrophages)
Lupus pernio=raised
indurated lesions on the cheeks, nose, ears
and forehead, may also be observed in
sarcoidosis
What is Lofgren syndrome
fever
erythema nodosum
bilateral hilar lymphadenopathy
polyarthralgia.
This is an acute presentation of sarcoidosis and has a
good prognosis. Treat with bed rest and NSAIDs.
Investigation of sarcoidosis
CXR
Bloods: calcium, ESR, LFT
spirometry
tuberculin skin test=negative (Mantoux test, used for TB diagnosis)
Management of sarcoidosis
- asymptomatic with bilateral lymphadenopathy=observe
- symptomatic stage 2,3,5=oral/inhaled corticosteroid. Cytotoxics (methotrexate or hydroxychloroquine) if sever or unresponsive
- Topical corticosteroid for cutaneous manifestation
mild=Hydrocortisone
Moderate=Eumovate
Potent=Betnovate
Potent with antimicrobials=fucibet
Stage 1 sarcoidosis CXR findings
bilateral hilar lymphadenopathy
Stage 2 sarcoidosis CXR findings
bihilar lymphadenopathy and reticulonodular infiltrates of parts or the entire lung.
Stage 3 sarcoidosis CXR findings
bilateral pulmonary infiltrates without signs of hilar lymphadenopathy
Stage 4 sarcoidosis CXR findings
fibrosis
What is acute respiratory distress syndrome (ARDS)
refers to non-cardiogenic
pulmonary oedema and lung inflammation that leads
to severe respiratory difficulty
what is a cause of ARDS
Sepsis
aspiration
pancreatitis
DIC
though to occur due to leakage of protein-rich fluid into alveolar–>inflammation–>hypoxia–>respiratory distress
What is the diagnostic criteria of ARDS (Berlin criteria)
- Dyspnoea and ARDS is acute
- Bilateral infiltrates on CXR
- PaO2:FiO2 ratio
201–300 (mild), 200–100 (moderate), <100
(severe) - Non-cardiogenic pulmonary oedema
- Pulmonary wedge pressure (PWP)= a measure of left arterial pressure.
An elevated PWP suggests
cardiogenic pulmonary oedema, while a lower
PCWP may help indicate ARDS.
Investigation in ARDS
CXR
Blood/sputum sample
urine culture (Sepsis)
echocardiogram (normal in ARDS)
What is PCWP used to determine. as in gold standard
PCWP is considered the gold standard for determining the cause of acute pulmonary edema; this is likely to be present at a PWP of >20mmHg.
what is the normal range of PCWP?
2-16mmHg
management of ARDS
low tidal volume ventilation
proning the patient, alters chest wall mechanics
sedation to minimise patient/ventilator dyssynchrony, especially in those with hypoxia.
what is extrinsic allergic alveolitis -EAA (hypersensitivity pneumonitis)
EAA is granulomatous inflammation
occurs in the lung due to inhaling organic or chemical
antigens or proteins.
what is are the different types of extrinsic allergic alveolitis
Farmer’s lung-mouldy hay-Saccharopolyspora rectivirgula
Bird fancier’s lung-protein in bird droppings
Malt worker’s lung- mouldy malt-Aspergillus
clavatus
Mushroom worker’s lung
Clinical features of EAA
expectorant cough
SOB
May present up to 8 hours after exposure
recover within a week.
Management of EAA
supportive-most resolve within a week
remove offending antigen, change of occupation
What is pneumoconiosis?
coniosis=Any of various diseases or pathological conditions caused by dust
Coal workers lung.
it is exemplified by fibrosis
that occurs secondary to repeated inflammation
caused by silica
Which occupation causes silicosis?
bricklaying, tunnelling, pottery and ceramic i
What is the difference between the cause of pneumoconiosis and silicosis?
pneumoconiosis= coal worker
silicosis= can by due to coal work but also bricklaying , pottery and ceramic.
What is Caplan syndrome
Coal worker’s pneumoconiosis with
associated rheumatoid arthritis
management of pneumoconiosis
Both social and emotional support
should be given to these patients. Coal worker’s
pneumoconiosis is a notifiable disease. Silicosis and
pneumoconiosis unfortunately have no specific therapy,
but symptomatic management should be offered.
Is pneumoconiosis and silicosis notifiable?
YES
What occupations are associated with asbestos?
work at shipyards, train
lines and in the plumbing industries
Asbestosis
What can exposure of asbestos cause?
lung cancer, specifically mesothelioma (cancer of the pleura)
management of asbestosis
There is
no specific therapy available. Treatment should focus
on minimising further exposure to asbestos, as well
as smoking cessation and managing any other co-morbid lung disease.
What is the difference between asbestosis and mesothelioma?
Asbestosis and mesothelioma are both diseases caused by asbestos exposure, but they are not the same. The primary difference is that asbestosis is not cancerous and is limited to the lungs and respiratory tract. Mesothelioma is an incurable cancer that develops in mesothelial tissue, typically in the lungs and abdomen.
investigation of asbestos related interstitial lung diseas?
CXR
CT chest (high res, contrast)
pleural biopsy
Management of both asbestosis and mesothelioma?
Treatment is supportive, with pleural surgery being
indicated for palliative symptomatic relief
Patients who develop asbestos-related disease may
be eligible for financial compensation, and it may be
helpful to discuss this with them.
