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Respiratory Pathology > Restrictive (Interstitial) Lung Diseases > Flashcards

Restrictive (Interstitial) Lung Diseases Flashcards

1
Q

What is the interstitium of the lung?

A

The connective tissue space around the airways and the vessels and the space between the basement membranes of the alveolar walls

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2
Q

Features of restrictive, diffuse interstitial lung disease

A

Reduced lung compliance - stiff lungs
Low FEV1 and Low FVC but FEV1/FVC normal ratio
Reduced gas transfer (diffusion abnormality)
Ventilation/perfusion imbalance

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3
Q

Presentation of diffuse lung disease

A

Discovery on abnormal CXR
SOB on exertion or rest
Type I resp failure
HF

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4
Q

Pathology of parenchymal (interstitial) lung injury

A 
Acute response
- diffuse alveolar damage (DAD)
Chronic response
- Usual interstitial pneumonitis (UIP) 
- granulomatous responses (sarcoidosis, hypersensitivity pneumonitis)
- other patterns
Fibrosis or end stage honeycomb lung
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5
Q

What is diffuse alveolar damage associated with?

A 
Major trauma
Chemical injury / toxic inhalation 
Circulatory shock 
Drugs
Infection 
Autoimmune disease
Radiation
Idiopathic
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6
Q

What does idiopathic mean?

A

Without a known cause

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7
Q

Evolution of DAD

A 
Exudative stage ( up to 7 days following the injury)
- oedema
- hyaline membranes damage 
Proliferative stage (7 days onwards)
- interstitial inflammation 
- interstitial fibrosis
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8
Q

What does DAD stand for?

A

Diffuse alveolar damage

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9
Q

Histological features of DADs

A 
Protein rich oedema
Fibrin 
Hyaline membranes
Denuded basement membranes
Epithelial proliferation 
Fibroblast proliferation 
Scarring; interstitium and lung spaces
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10
Q

What is sarcoidosis?

A

A multisystem granulomatous disorder of unknown aetiology

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11
Q

Histology of sarcoidosis

A

Epithelioid and giant cell granulomas
Necrosis/caseation very unusual
Little lymphoid infiltrate
Variable associated fibrosis

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12
Q

Who gets sarcoidosis?

A

Young adults

F > M

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13
Q

Organ involvement in sarcoidosis

A 
Lymph nodes
Lung 
Spleen 
Liver
Skin, eyes, skeletal muscle
Bone marrow
Salivary glands
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14
Q

Presentation of sarcoidosis

A 
Acute arthralgia (polyarthralgia)
Erythema nodosoum 
Bilateral hilar lymphadenopathy 
Swinging fever 
Asymptomatic - accidental abnormal CXR
SOB 
Cough
Lupus pernio 
Hypercalcaemia
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15
Q

Treatment of sarcoidosis

A

Corticosteriods

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16
Q

Diagnosis of sarcoidosis

A 
Clinical findings
Imaging
Serum calcium +++ and ACE
CXR
PFTs
Biopsy 
Bloods / urinalysis / ECG/ Eye exam 
Bronchoscpy including biopsies 
Kveim test
Tuberculin test
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17
Q

Antigens in hypersensitivity pneumonitis

A

Thermophilic actinomycetes

  • micropolyspora faeni
  • thermoactinomyces vulgaris
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18
Q

Presentation of hypersensitivity pneumonitis

A 
Acute presentation 
- fever
- dry cough 
- myalgia 
- chills 4 - 9 hours after Ag exposure 
- crackles
- tachypnoea
- wheeze
Chronic presentation 
- insidious
- malaise
- SOB
- cough 
- low grade illness 
- crackles and some wheeze
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19
Q

Does acute hypersensitivity pneumonitis have a precipitating antibody?

A

Yes

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20
Q

What can hypersensitivity pneumonitis lead to?

A

Resp failure

Gas transfer low

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21
Q

Pathology / histology of hypersensitivity pneumonitis

A 
Immune complex mediated combined Type III and Type IV hypersensivity reaction 
Soft centraacinar epitheliod granulomata
Interstitial pneumonitis
Foamy histocytes
Bronchiolitis obliterans
Upper zone disease
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22
Q

What may unusual interstitial pneumonitis (UIP) be seen in?

A

Connective tissue diseases; especially scleroderma and rheumatoid disease
Drug reaction
Post infection
Asbestos exposure
Cryptogenic or idiopathic (idiopathic pulmonary fibrosis (IPF) and cryptogenic fibrosing alveolitis (CFA)

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23
Q

Histology of UIP

A

Patchy interstitial chronic inflammation
Type II pneumocyte hyperplasia
Smooth muscle and vascular proliferation
Evidence of old and recent injury
- temporal heterogenicity
- spatital heterogenicity
Proliferating fibroblastic foci

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24
Q

Presentation of idiopathic pulmonary fibrosis

A 
Clinical syndrome
SOB (chronic)
Cough (chronic)
Basal crackles
Cyanosis 
Pneumonitis 
Clubbed or crackles 
Progressive disease; most dead within 5 years
Restrictive PFT and reduced gas transfer
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25
Q

Who gets idiopathic pulmonary fibrosis?

A

60 - 70 y/o

M > F

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26
Q

What would a CXR of idiopathic pulmonary fibrosis show?

A

Basal / posterior
diffuse infiltrates
Cysts
“Ground glass”

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27
Q

Prognosis of idiopathic pulmonary fibrosis

A

Poor

  • some fulminant
  • some steroid response
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28
Q

What happens beyond the terminal bronchiole?

A

Diffusion

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29
Q

Normal Pa02

A

10.5 - 13.5 kPa

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30
Q

Normal PaCO2

A

4.8 - 6.0 kPa

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31
Q

What is type I Resp failure?

A

PaO2 < 8kPa (PaCO2 normal or low)

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32
Q

What is type II resp failure?

A

PaCO2 > 6.5kPa (Pa02 usually low)

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33
Q

What are the 4 abnormal states Assosiated with hypoxaemia?

A

Alveolar hypoventilation
Shunt
Ventilation/perfusion imbalance (V/Q)
Diffusion impairment

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34
Q

Pathology of alveolar hypoventilation

A

Hypoventilation increases PaCO2 and thus increases PaCO2
Increased in PaCO2 decreases Pa02 which causes Pa02 to fall
Fall in PaO2 due to hypoventilation is corrected by raising FIO2.

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35
Q

What is FIO2?

A

Fraction of inspired air which is oxygen

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36
Q

Normal V/Q

A

4/5 or 0.8

37
Q

What is the commonest cause of hypoxaemia encountered clinically?

A

Low V/Q

38
Q

Why does Low V/Q in some alveoli airse?

A

Due to local alveolar hypoventilation due to some focal disease

39
Q

What does hypoxaemia due to low V/Q respond well to?

A

Even small increases in Fi02

40
Q

What does gas flow through a membrane depend on?

A

Thickness and surface areas of membrane

Gas pressure across it

41
Q

CO2 diffusion vs O2 diffusion

A

CO2 20x faster due to greater solubility

42
Q

Do diseases impairing gas diffusion usually change CO2 levels?

A

No

43
Q

What does diffusion impairment lead to?

A

Longer for blood and alveolar air to equibrillate, particularly for O2

44
Q

How long does equilibrillation usually take?

A

0.25 seconds

45
Q

What is the capillary transit time?

A

0.75 seconds

46
Q

What can correct hypoxaemia?

A

Increasing FiO2

47
Q

What is a shunt?

A

Blood passing from R to L side of the heart WITHOUT contacting ventilated alveoli

48
Q

When do you get pathological shunts?

A

AV malformations
Congenital heart disease
Pulmonary disease

49
Q

What is the physiological definition of restriction?

A

FVC < 80% of the predicted normal

50
Q

What is FVC affected by normally?

A

Age
Size
Ethnicity

51
Q

What is the marker of restriction?

A

Vital capacity

52
Q

Possible systems causing restriction

A
  1. Lungs
  2. Pleura
  3. Nerve or muscle
  4. Bone
  5. Other
53
Q

Lung causes of restriction

A

Interstitial lung disease

  • Idiopathic pulmonary fibrosis
  • sarcoidosis
  • hypersensitivity pneumonitis
54
Q

Pleural causes of restriction

A

Pleural effusions
Pneumothorax
Pleural thickening

55
Q

What is a pleural effusion?

A

Fluid in pleural space

56
Q

What is a pneumothorax?

A

Air in pleural space

57
Q

Skeletal causes of restriction

A

Kyphoscoliosis
Ankylosing spondylitis
Thoracoplasty (elderly)
Rib fractures

58
Q

Muscle causes of restriction

A

Amyotrophic lateral sclerosis

59
Q

Sub diaphragmatic causes of restriction

A

Obesity

Pregnancy

60
Q

What occurs in the interstitium?

A

Gas diffusion

61
Q

What are interstitial lung diseases?

A

> 200 diseases causing thickening of the interstitium and can result in pulmonary fibrosis

62
Q

What are the top 3 interstitial lung diseases?

A

Sarcoidosis
Idiopathic pulmonary fibrosis
Hypersensitivity pneumonitis

63
Q

What is sarcoidosis?

A

Multisystem granulomatous disease of unknown aetiology

64
Q

What is the histological hallmark of sarcoidosis?

A

Non caseating granuloma

65
Q

What is the most common interstitial lung disease?

A

Sarcoidosis

66
Q

Presentation of sarcoidosis

A 
Lung effects
Anterior / posterior uveitis 
Erythema lodosum 
Pink lesions 
Skin manifestation of cardiod
67
Q

Stages of sarcoidosis (pulmonary)

A

Stage 1 - 4

68
Q

Remission rate of each stage of sarcoidosis in the lungs based on CXR

A

Stage 1 - 55-90%
Stage 2 - 40 - 70%
Stage 3 - 10-20%
Stage 4 - 0%

69
Q

Treatment of sarcoidosis with mild disease no vital organ involvement, normal lung function and few symptoms

A

NO treatment

70
Q

Treatment of sarcoidosis if have erythema nodosum / arthralgia

A

NSAIDs

71
Q

Treatment of sarcoidosis if skin lesions / anterior uveitis / cough

A

Topical steroids (inhalers / drops / cream)

72
Q

Treatment of sarcoidosis if cardiac, neurological, eye disease not responding to topical treatment or hypercalcaemia

A

Systemic steroids

73
Q

What % of sarcoidosis patients sustain permanent or extra pulmonary complications?

A

10 - 20%

74
Q

Pulmonary Complications of sarcoidosis

A

Progressive resp failure
Bronchiectasis
Aspergilloma, haemoptysis
Pneumothorax

75
Q

What do a lot of patients with idiopathic pulmonary fibrosis have previous problems of?

A

HF / heart problems

76
Q

Median survival of idiopathic pulmonary fibrosis

A

3 years

77
Q

Treatment of idiopathic pulmonary fibrosis

A 
Oral anti fibrotic
Pirfendone
Nintedanib 
Double lung transplant (consider < 65 y/o)
Palliative care
78
Q

Common occupation / hobbie when dealing with hypersensitivity pneumonitis

A

Bird feather dust
Farmers - mould and dusty hay
Malt workers lung

79
Q

What zones of the lungs does sarcoidosis cause fibrosis in?

A

Upper lobes

80
Q

Investigation for idiopathic pulmonary fibrosis

A

High resolution CT

81
Q

Causes of pulmonary fibrosis of the upper zones

A 
Hypersensitivity pneumonitis (extrinsic allergic alveolitis)
Coal workers pneumoconiosis 
Silicosis
Sarcoidosis
AS (rare)
Histiocytosis 
TB
82
Q

Causes of pulmonary fibrosis of the lower zones

A 
Idiopathic pulmonary fibrosis
Most connective tissue disorders (except AS) e.g. SLE
Drug induced
- amiodarone
- methotrexate 
Asbestosis
83
Q

What is lupus pernio? What does it look like?

A 
Cutaneous manifestation of sarcoidosis 
Most frequently affects 
- cheeks
- nose
- cheeks
- lips
- ears
- digits 
Not painful or itchy
84
Q

What diagnosis would a facial rash and lymphadenopathy make you think of?

A

Sarcoidosis

85
Q

Presentation of idiopathic pulmonary fibrosis

A

Progressive exertional SOB
Bibasal fine end inspiratory creps on auscultation
Dry cough
Clubbing

86
Q

What is raised in sarcoidosis?

A

ACE levels

87
Q

What can acute sarcoidosis mimic?

A

Other types of arthritis

88
Q

Indications for corticosteroid treatment in sarcoidosis

A

Parenchymal lung disease
Uveitis
Hypercalcaemia
Neurological or cardiac involvement

Respiratory Pathology (16 decks)

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