Interstitial lung disease Flashcards by Miranda Pearson

What is ILD?

lung disease associated with interstitium , tissue around the alveoli

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what is the most common ILD type?

idiopathic pulmonary fibrosis

others: systemic sclerosis, Lupus

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what is idiopathic pulmonary fibrosis?

progressive scarring and fibrosis of the lung interstitium

no discernible underlying cause

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what drugs (name 3 ) that can cause IPF?

methotrexate, amiodarone, bleomycin

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IPF is common over what age group?

> 60 yrs

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Is there ethnic factors involves in IPF?

no, but a familial variant (FPF, Familial Pulmonary Fibrosis) exists

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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

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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

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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

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What is sarcoidosis

chronic granulomatous disorder of unknown cause

multi-organ system disorder

histologically=non-caseating granulomas

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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

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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

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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.

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Investigation of sarcoidosis

CXR
Bloods: calcium, ESR, LFT
spirometry
tuberculin skin test=negative (Mantoux test, used for TB diagnosis)

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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

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Stage 1 sarcoidosis CXR findings

bilateral hilar lymphadenopathy

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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.