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Respiratory > Restrictive lung disease > Flashcards

Restrictive lung disease Flashcards

1
Q

What is the pathology behind restrictive lung disease

A

Pathological material enters
Acute inflammation = diffuse alveolar damage (DAD)
Leads to massive pulmonary oedema
Cells are damaged
Vessels become more leaky
Macrophages and proteins form hyaline membranes
Interstitial inflammation and fibrosis = rapid
Can be fatal

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2
Q

What can DAD also be

A

ARDS

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3
Q

What are causes - ask in HX

A 
Occupational exposure
Drugs -  amiadarone 
Hypersensitivity to allergens - EAA 
Infection - TB / fungi / pneumonia 
GORD associated
Systemic causes
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4
Q

What are systemic causes - ask in Hx

A 
Sarcoidosis
SLE
Systemic sclerosis
RA 
UC
Vasculitis 
Autoimmune thyroid
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5
Q

What if unknown cause

A

Known as idiopathic pulmonary fibrosis

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6
Q

What are symptoms of restrictive lung disease and what does it lead too

A 
SOB
Dry cough
Crackles - end of inspiration 
Abnormal breath sounds
Shallow breath as reduced chest size 
Abnormal CXR / CT
Clubbing 
Type 1 resp failure
Pulmonary hypertension -> 
Cor pulmonale
HF
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7
Q

What are RF for developing

A 
Major trauma
Chemical injury
Circulatory shock
Drugs
Infection
Autoimmune
Radiation
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8
Q

What is investigation of choice for DADs

A

CXR - show shaggy heart / diaphragm (diff oedema)
Always
HRCT

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9
Q

What does histology show

A 
Protein rich oedema
Hyaline membrane
Loss of fibrin
Epithelial fibroblast proliferation 
Scarring
Fibrosis + remodelling of interstitium
Chronic inflammation
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10
Q

Is there reduced lung compliance

A

Yes as stiff

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11
Q

What does spirometry show

A 
Low FEV!
Low FVC
Ratio the same
Reduced gas transfer as diffusion abnormality 
V/Q imbalance
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12
Q

What causes chronic

A

Sarcoidosis
UIP - usual interstitial pneumonia
Granulomatous
Hypersensitivity pneumonitis

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13
Q

What is sarcoidosis

A

Multisystem granulomatous disorder
Granuloma = inflammatory cluster filled with mostly macrophages
Non-ceasaing = no necrosis and is buzzword for sarcoidosis

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14
Q

What is histology in sarcoidosis

A 
Epithelioid and giant cell granuloma's
Filled with MO 
Little lymphoid infiltrate
Variable fibrosis
Alveoli surrounding granuloma = normal
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15
Q

What causes sarcoidosis

A

Unknown cause
Immune reaction to unknown antigen?
Can affect any organ

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16
Q

What does acute sarcoid present like

A

Bilateral hilar lymphadenopathy - seen on CXR or CT
Swinging fever
Erythema nodosum
Polyarthralgia

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17
Q

When is bilateral hilariously lymphadenopathy also common

A 
TB
Malignancy - lymphoma 
Fungi
HIV 
Mycoplasma 
Hypersensitivty pneumonitis 
Histiocytosis
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18
Q

What are insidious symptoms

A 
SOB
Dry cough 
Crackles 
Chest pain
Malaise
Weight loss
Pink lesions containing granuloma - over scar tissue
Clubbing
Mediastinal LN
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19
Q

What are skin symptoms

A

Lupus pernio

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20
Q

Symptoms of non-pulmonary disease

A 
Systemic - fever, fatigue, weight loss
Hypercalcaemia - macrophages in granuloma increase conversion from vitae's D
Lymphopenia
LN
Liver cirrhosis / cholestasis 
HSM
Uveitis
Conjunctivitis
Optic neuritis
Bells Palsy
Neuropathy
Cardiomyopathy 
Heart block 
Renal
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21
Q

Who is affected by sarcoid

A

Young females

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22
Q

How do you Dx

What is diagnostic

A

Blood - Elevated serum Ig / ACE / Ca / LFT / ESR
CXR
Spirometry = restrictive
Transbronchial biopsy / bronchoscopy = diagnostic
Mediastinoscopy + biopsy of hilar LN

Other organ involvement 
U+E / urine dip 
LFT
ECHO - if cardiac
ECG- BBB/. arrhythmia 
PET CT to see what lights up 
TB test
USS abdo
Examine eye for occular
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23
Q

What does CXR show

A 
Trachea
Nodes
Chunky light hilar
Pulmonary infitrates
Fibrosis
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24
Q

What does biopsy show

A

Non-caesating granuloma

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25
Q

How do you treat mild disease, normal PFT, no important organ

A

No treatment

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26
Q

What do you do if erythema nodosum / arthralgia

A

NSAID

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27
Q

What do you do if skin lesions / uveitis / cough

A

Topical Steroid

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28
Q

Cardia / neuro / hypercalcaemia / symptomatic stage 2/3 disease

A

Systemic steroid
Biphosphonates for hypercalcaemia
2nd line = immunosuppression

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29
Q

What is stage 1 on CXR

A

Bilateral hilar lymphadenopathy

30
Q

Stage 2

A

+ pulmonary infiltrate

31
Q

Stage 3

A

Pulmonary infiltrates ONLY

32
Q

Stage 4

A 
Fibrosis
Causes pulmonary hypertension 
Loss of lung function
Impairment to lung function / gas exchange
Benefit from lung transplant
33
Q

What are complications of sarcoidosis

A 
Respiratory failure 
Bronchiectasis
Aspergilloma
Haemoptysis
Pneumothorax
34
Q

DDX of sarcoidosis

A

Infection
Autoimmune
Vasculitis
EAA

35
Q

What is hypersensitivity pneumonitis also known as

A

Extrinsic allergic alveolitis

36
Q

What is hypersensitivity pneumonitis

A

Group of diseases caused by inhalation of allergen

Immune mediated

37
Q

What type of hypersensitivity and histology

A 
Type 3 = immune complex
Granuloma's - centracinar (type 4) 
Foamy histiocytes
Intersitital pneumoinitis
Bronchiolitis obliterans can occur
38
Q

What happens in acute

A

Alveoli infiltrated by inflammatory cells

If remove allergen process can be halted

39
Q

What happens in chronic

A

Granulomas + lymphocytes = chronic inflammation
Fibrosis
Bronchiectasis

40
Q

What are acute symptoms 4-8 hours after exposure

A 
Recurrent SOB
Dry cough 
Pyrexia
Myalgia
Bibasal crackles
Wheeze
Tachypnoea
Flu symptoms
Reduction in lung volume and diffusion
41
Q

What are chronic symptoms

A 
Fibrosis
Increasing SOB
Weight loss
Cough
Crackles
Wheeze
Malaise / lethargy 
Clubbing
Type 1 resp failure
Cor pulmonale
42
Q

How do you investigate

A

CXR
BAL
Biopsy
FBC - increased WCC, neutrophil, ESR

43
Q

What does CXR show

A

Fibrosis of upper and middle lobe

Diffuse shadowing

44
Q

What does biopsy show

A

Fibroses
Alveolitis
Granuloma

45
Q

What does BAL show

A

Lymphocytosis and mast cells

46
Q

How do you treat

A

Remove allergen
Steroids in severe
Oxygen may be used in acute attacks

47
Q

Often misDx as

A

Pneumonia

48
Q

What is Ddx of fibrosis

A

Infection
Lymphoma
Carcinoma
Haemorrhage

49
Q

What is idiopathic pulmonary fibrosis

A

Most common cause of fibrosis in the lung

50
Q

Pathology of IPF

A 
Patchy chronic inflammation
Intra-alveolar fibrosis
Obliteration of bronchioles
Smooth muscle and vascular proliferation
Turns into collagen
51
Q

What causes IPF

A

Connective tissue - ANCA / RF +Ve
Drugs
Asbestos
After viral infection

52
Q

Symptoms of IPF

A

Progressive exertional dyspnoea
Dry cough
Basal fine crackles at end of inspiration
Clubbing

Malaise
Fever
Weight loss
Arthralgia
Elevated ESR
Cyanosis
Failed Rx of LVF / pneumonia
53
Q

How do you investigate

A 
ABG - decreased O2
Bloods -  increased CRP, Ig, ANCS, RF
CT = diagnostic
CXR
Spirometry - restrictive
BAL
PFT - reduced gas transfer
54
Q

What does CXR show

A 
Fleeting shadows
Small irregular opacities
Peripheral opacities -> honeycomb late 
Diaphragm and fuzzy heart border
Smaller lung volume 
Often misdiagnosed as pneumonia
55
Q

What does BAL show

A

Lymphocytosis
Neutrophils
Eosinophilia suggest worse prognsosi

56
Q

How do you treat

A 
Often poor response to Rx
Pulmonary rehab
Oral anti fibrotic
Supplementary O2
Opiates for SOB 
Palliative
Surgical transplant
57
Q

What are the complications

A 
Lung cancer
Fibrosis
Honeycomb
End stage lung disease
Cor pulmonale and res failure = death
58
Q

What are most common restrictive lung disease

A

Idiopathic
Drugs
LL tend to be affected

59
Q

What affects upper lobe

A 
CHARTS 
Coal work - hypersnesitivity
Histiocytosis / hypersnesitivity / EAA 
AS 
Radiation
TB
SArcoidosis
60
Q

What affects LL

A 
Idiopathic
Connective tissue
Drugs 
Asbestos
a!A!
61
Q

What is the advanced stage of fibrosis

A

Honeycomb lung

62
Q

What drugs cause fibrosis

A

Amiadarone
Cyclophosphomdie
Methotrexate
Nitrafuratoin

63
Q

What is hypoxia at rest an indication for

A

Oxygen therapy

64
Q

What can be given to slow down progression

A

Anti-fibrinolytic

65
Q

To get drug what is required

A

Non-smoker

FVC <80%

66
Q

What lifestyle

A

Smoking cessation

Pulmonary rehab

67
Q

What do yo follow up patients with

A

CXR

Spirometry and PFT

68
Q

What is important in history

A 
Childhood - TB / pneumonia
Occupational exposure
Dust / asbestos 
Immune - scleroderma / RA / sarcoid
Smoking
Drugs 
Pets
Feather duvet 
FH
69
Q

When should you think if ILD

A

Desaturations

Chronic SOB / chronic cough

70
Q

What is general management of ILD

A 
Remove cause 
Home O2 if hypoxic at rest
Stop smoking
Physio and pulmonary rehab 
Pneumococcal and flu 
Lung transplant is an option
ACP and palliation
71
Q

What type of crackles

A

Fine end inspiratory

72
Q

In pneumonia

A

Coarse inspiratory

Respiratory (23 decks)

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