Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

2. Respiratory Medicine (Lectures) > 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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

Presentation of diffuse lung disease

A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

What is diffuse alveolar damage associated with?

A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

What does idiopathic mean?

A

Without a known cause

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

What does DAD stand for?

A

Diffuse alveolar damage

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

What is sarcoidosis?

A

A multisystem granulomatous disorder of unknown aetiology

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

Histology of sarcoidosis

A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

Who gets sarcoidosis?

A

Young adults
F > M

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

Organ involvement in sarcoidosis

A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

Treatment of sarcoidosis

A

Corticosteriods

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

Antigens in hypersensitivity pneumonitis

A

Thermophilic actinomycetes
- micropolyspora faeni
- thermoactinomyces vulgaris

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

Does acute hypersensitivity pneumonitis have a precipitating antibody?

A

Yes

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

What can hypersensitivity pneumonitis lead to?

A

Resp failure
Gas transfer low

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
Q

Who gets idiopathic pulmonary fibrosis?

A

60 - 70 y/o
M > F

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
26
Q

What would a CXR of idiopathic pulmonary fibrosis show?

A

Basal / posterior
diffuse infiltrates
Cysts
“Ground glass”

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
27
Q

Prognosis of idiopathic pulmonary fibrosis

A

Poor
- some fulminant
- some steroid response

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
28
Q

What happens beyond the terminal bronchiole?

A

Diffusion

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
29
Q

Normal Pa02

A

10.5 - 13.5 kPa

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
30
Q

Normal PaCO2

A

4.8 - 6.0 kPa

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
31
Q

What is type I Resp failure?

A

PaO2 < 8kPa (PaCO2 normal or low)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
32
Q

What is type II resp failure?

A

PaCO2 > 6.5kPa (Pa02 usually low)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
33
Q

What are the 4 abnormal states Assosiated with hypoxaemia?

A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
35
Q

What is FIO2?

A

Fraction of inspired air which is oxygen

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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

2. Respiratory Medicine (Lectures) (50 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2024 Bold Learning Solutions. Terms and Conditions