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Respiratory > ILD, EAA, IPF > Flashcards

ILD, EAA, IPF Flashcards

1
Q

What is interstitial lung disease

A

Generic term used to describe a number of conditions that primarily affect the lung parenchyma in a diffuse manner

Chronic inflammation and/or progressive interstitial fibrosis

Share a number of clinical and pathological features

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2
Q

What are the clinical features of ILD

A

Dyspnoea on exertion
Non-productive paroxysmal dry cough

Abnormal breath sounds

Chest pain - increased risk of pneumothorax due to fibrosis

Clubbing

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3
Q

Pathological features of ILD

A

Chronic inflammation
Fibrosis and remodelling of interstitium
Hyperplasia of type II epithelial cells or type II pneumocytes

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4
Q

3 categories of ILD

A

Known Cause
Associated with systemic disease
Idiopathic

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5
Q

Idiopathic causes of ILD

A

Idiopathic pulmonary fibrosis
Cryptogenic organising pneumonia
Lymphocytic interstitial pneumonia

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6
Q

ILD associated with systemic disorders

A 
Sarcoidosis 
RA
SLE 
UC 
Connective tissue disease 
Autoimmune thyroid disease
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7
Q

Known causes of ILD

A 
Occupational/environmental 
Drugs 
Hypersensitivity reactions (EAA)
Infections eg. TB, fungal or viral 
GORD
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8
Q

ILD CXR

A

Patchy shadowing

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9
Q

HR CT ILD

A

Fibrosis

Honeycombing - IPF

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10
Q

What is Extrinsic Allergic Alveolitis

A

Inhalation of allergens (fungal spores or avian proteins) causes a hypersensitivity reaction in sensitised individuals

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11
Q

What happens in acute and chronic phase of EAA?

A

Acute - alveoli infiltrated with acute inflammatory cells

Chronic - granuloma formation and obliterative bronchiolitis

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12
Q

Causes of EAA - the sensitizing thing

A

Bird fanciers and pigeon fanciers lung - proteins in bird droppings

Farmers and mushroom workers lung

Malt workers lung

Sugar workers lung

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13
Q

Clinical features 4-6 hour post exposure to allergen in EAA

A

Fever, rigours, myalgia, dry cough, dyspnoea, crackles but no wheeze
Inspiratory crepitations

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14
Q

Chronic features of EAA

A 
Increasing dyspnoea and decreasing exercise tolerance 
Weight loss
type 1 respiratory failure 
cor pulmonale 
Inspiratory crepitations
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15
Q

CXR in acute and chronic EAA

A

ACUTE
Upper-zone mottling/consolidation

CHRONIC
upper lobe fibrosis
Honeycomb lung

CT
Ground glass appearance

Bronchoalveolar lavage - CD8 T-lymphocytes and mast cells

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16
Q

Lung function tests in EAA

A

Restrictive - therefore FVC decrease more than FEV1

Decreased total lung capacity

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17
Q

Management of EAA acutely

A

Remove allergen and give O2 - acutely

Then oral prednisolone

18
Q

Long term managment of EAA

A

Avoid exposure to allergens
Wear a facemask or +ve pressure helmet if unavoidable

Long-term steroids may improve CXR and physiology but not always - 1month trial

Compensation?!?

19
Q

Causes of upper zone fibrotic shadowing on CXR

A 
TB
EAA
Ankylosing spondylitis 
Radiotherapy 
Sarcoid
20
Q

Causes of middle zone fibrotic shadowing on CXR

A

Progressive massive fibrosis

21
Q

Causes of lower zone shadowing on CXR

A

IPF

Asbestosis

22
Q

What are the 5 ways that the aspergillus fungi affect the lung

A

1) Asthma - type 1 hypersensitivity reaction to fungal spores
2) Allergic bronchopulmonary aspergillosis
3) Aspergilloma
4) Invasive aspergillosis
5) Extrinsic Allergic alveolitis - ‘malt workers lung’ sensitivity to this fungi

23
Q

What is allergic bronchopulmonary aspergillosis?

A

Hypersensitivity to aspergillus fungus
Causes bronchoconstriction but then inflammation and permanent damage occurs causing bronchiectasis
Wheeze, cough, sputum, dyspnoea and ‘recurrent pneumonia’

24
Q

Treatment of allergic bronchopulmonary aspergillosis ABPA

A

Prednisolone for acute attacks 30-40mg
Maintenance dose of 5-10mg
Sometimes itraconazole

25
Q

What is aspergilloma?

A

Fungal ball within a pre-existing cavity (TB or sarcoid)
Usually asymptomatic
Can cause cough with haemoptysis
Also lethargy and weight loss
Treatment is removal if solitary symptomatic or lots of haemoptysis

26
Q

What is invasive aspergillosis?

A

Invasion of aspergillus into lung tissue and fungal dissemination to other parts of the body in immunocompromised patients
dyspnoea, rapid deterioration and septic picture
Treat with voriconazole or other -zole’s

27
Q

What is Idiopathic Pulmonary Fibrosis?

A

Type of idiopathic interstitial lung disease
Inflammatory cell infiltrate and lung fibrosis - unknown cause
Also called idiopathic fibrosing alveolitis and previously called cryptogenic fibrosing alveolitis

28
Q

Signs of IPF

A

Dry cough, no wheeze, gradual onset of exertional dyspnoea, clubbing, cyanosis, fine end-inspiratory creps
Malaise + fatigue, weight loss and arthralgia

29
Q

Complications associated with IPF

A

Resp. failure and increased risk of lung cancer

30
Q

Bloods found in IPF

A

ANA 30% +ve
Rheumatoid factor in 10%
Immunoglobulins raised

31
Q

Bronchoalveolar lavage with IPF

A

Neutrophils and lymphocytes

32
Q

CXR in IPF

A

Decreased lung volume
ground glass
Bilateral lower zone reticulonodular shadows
Honeycomb lung

33
Q

Management of IPF

A

O2 pulmonary rehab, opiates and palliative care
Not high-dose steroids
Trial 3-6months of azathioprine, oral glucocorticoids and high-dose acetylcysteine

34
Q

What is coal workers pneumoconiosis

A

Dust disease due to inhalation of coal particles in mines over 15-20 years
Ingested by macrophages which die and release enzymes causing fibrosis
Asymptomatic but can get bronchitis
CXR - opacities - especially in upper zone
Usually progressive onset of SOB and dry cough - can get black sputum, melanoptysis

35
Q

What is progressive massive fibrosis

A

Progression of CWP - progressive dyspnoea, fibrosis and eventually cor pulmonale
CXR - upper zone fibrotic masses

36
Q

What is Caplan’s syndrome?

A

Association of RA, pneumoconiosis and pulmonary rheumatoid nodules

37
Q

What is silicosis

A

Caused by inhalation of silica particles
Very fibrogenic
Progressive dyspnoea
Upper and middle zone nodular pattern

38
Q

What is asbestosis

A

Inhalation of asbestos fibres (blue worse than white) causing fibrosis
Progressive dyspnoea, clubbing, fine end-inspiratory crackles
Increased risk of bronchial adenocarcinoma and mesothelioma

39
Q

What is malignant mesothelioma

A

Tumour of mesothelial cells in pleura
Complex relationship with asbestos exposure - not all have pulmonary asbestosis

Chest pain, dyspnoea, weight loss, clubbing, recurrent pleural effusions
Metastases

40
Q

Management of malignant mesothelioma

A

Pemetrexed+cisplatin chemotherapy

Pleurodesis or indwelling drain may help

Respiratory (7 decks)

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