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RESPIRATORY > Interstitial Lung Disease > Flashcards

Interstitial Lung Disease Flashcards

1
Q

what is extrinsic allergic alveolitis

A

hypersensitivity pneumonitis
Chronic inflammatory disease as a result of intersitial lung disease
in Small airways, intersitium
occasionally granulomas

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2
Q

what type of hypersenstivity reaction is extrinsic allergic alveolitis

A

Type III- immune complex deposition reaction to antigen
lymphocytic alveolitis
combination type III and type IV

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3
Q

what type of people get extrinsic allergic alveolitis and what causes them to be infected

A

farmers lung, mushroom workers = thermophilic actinomycetes

malt workers = fungi

bird fanciers lung = avian antigens eg. pigeon exposure (delayed hypersistivity), budgies (exposure over time)

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4
Q

what drugs can cause extrinsic allergic alveolitis

A

gold, bleomycin, sulphasalazine

gold was used in rheumatology

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5
Q

what percentage of causes of extrinsic allergic alveolitis is no cause identified

A

30%

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6
Q

how is extrinsic allergic alveolitis diagnosed

A

precipitins (antibodies) in serum

biopsy

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7
Q

acute features of extrinsic allergic alveolitis

A

cough, breathless, fever, myalgia

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8
Q

a bird fancier tells you that they are fine when in contact with the birds and cleaning them out but hours later experience cough, breathlessness and fever (flu-like), what is the diagnosis

A

extrinsic allergic alveolitis

delayed hypersensitivity

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9
Q

what are the signs of acute extrinsic allergic alveolitis

A

+/- pyrexia
crackles, NO WHEEZE
hypoxia

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10
Q

why is bird fanciers lung dangerous

A

can go into pulmonary failure

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11
Q

how would a bird fanciers lung show on x-ray

A

widespread pulmonary infiltrates

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12
Q

treatment of bird fanciers lung (avian antigens)

A

oxygen
steroids
antigen avoidance

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13
Q

what type of people experience chronic extrinsic allergic alveolitis

A

people who keeps parrots, budgies or ride horses experience repeated low dose antigen exposure over time (years)

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14
Q

what are the signs of chronic extrinsic allergic alveolitis

A

crackles
progressive breathlessness and cough
PFTs: restrictive defect - low FEV1 and FVC, high/normal ratio and low gas transfer - TLCO

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15
Q

how would you diagnose chronic extrinsic allergic alveolitis

A

history of exposure
precipitins (IgG antibodies to antigen)
lung biopsy if in doubt
x-ray: pulmonary fibrosis, more common in upper zones

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16
Q

how is chronic extrinsic allergic alveolitis treated

A

remove antigen exposure

oral steroids if breathless/low gas transfer for ongoing inflammation

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17
Q

what is the most important/common type of interstitial lung disease

A

idiopathic pulmonary fibrosis

as Cryptogenic Fibrosing Alveolitis

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18
Q

what is the cause of idiopathic pulmonary fibrosis

A

unknown
imbalance of fibrotic repair system: lungs start to repair themselves when they do not need repaired,  Multiple micro injuries to alveolar cells cause them to secrete growth factors that recruit fibroblasts -> myofibroblasts which aggregate and fibrotic foci

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19
Q

is there inflammation present in idiopathic pulmonary fibrosis

A

no

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20
Q

what group of people are at higher risk of IPF

A

smokers

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21
Q

causes of IPF

A

rheumatoid, SLE, systemic sclerosis, asbestosis

drugs - amiodarone, busulphan, penicillamide, nitorfurantoin (given to elderly with UTI), methotrexane

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22
Q

risk factors of IPF

A 
	Smoking 
	Chronic aspration
	Antidepressants
	Wood + metal dusts 
	Infection - Epstein-Barr virus
	Autoimmune disease
more common in males
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23
Q

what is the pathology of IPF

A

Usual Interstitial Pneumonia pattern (UIP) heterogenous fibrosis in alveolar walls with fibroblastic foci and destruction of architecture causing honeycombing.

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24
Q

a patient presents with progressive breathlessness over several years, dry cough, cyanosis and on examination have finger clubbing and bilateral fine inspiratory crackles, what is the diagnosis

A

Idiopathic pulmonary fibrosis

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25
Q

if untreated what does IPF lead to

A

resp failure
pulmonary hypertension
cor pulmonale

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26
Q

is IPF restrictive or obstructive lung disease

A

restrictive
reduced FEV1 and FVC with normal/raised FEV1/FVC ratio
reduced lung volumes
low gas transfer

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27
Q

a patient chest x-ray shows bilateral infiltrate, ground glass appearance, irregular reticlonodular shadowing in lower zones and Honeycomb lung what is a possible diagnosis

A

IPF

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28
Q

what does a patient which IPF show on CT scan

A

reticulonodular fibrotic shadowing, worse at the lung bases, and periphery (gradually grows inwards)
honeycombing - cystic changes
patchy fibrosis of intersitium, subpleura and paraseptal
acute fibroplastic proliferation
collagen deposition

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29
Q

what test can be carried out if unsure IPF after chest x-ray and HRCT scan

A

lung biopsy - trans bronchial or throacicscopic

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30
Q

describe the blood gasses of a patient with IPF

A

arterial hyperaemia due to alveolar-capillary block

ventilation-perfusion mismatch with normal/low PaCO2 due to hyperventialtion

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31
Q

what would a blood test of a patient with IPF show

A

anti-nuclear antibodies
rheumatoid factors
ESR + antibodies = elevated

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32
Q

there would be increased/decreased neutrophils and macrophages in bronchoalveolar lavage of a patient with IPF

A

increased

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33
Q

what is the treatment of IPF

A

best supportive care
steroids/immunosuppresants does not reverse fibrosis but can slow progression
transplant in young patients <60

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34
Q

what can be given in an attempt to disable IPF

A

prednisolone

azathioprine/cyclophosphamide added if no response

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35
Q

what new drugs are in trial to treat IPF

A

Prifenidone and Nintadanib
side effects: diarrhoea, nausea, sun sensitivity
very expensive

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36
Q

is IPF usually a disease of younger or older people

A

older >70

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37
Q

what is the prognosis of IPF

A

most patients progress into resp failure in first few years

4 year survival from point of diagnosis

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38
Q

what is another name of diffuse parenchymal lung disease

A

restrictive thoracic disease -

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39
Q

what is DPLD

A

the volume of the lung shrinks interrupting of the alveolar interior barrier so there is a ventilation-perfusion mismatch
harder for gases to enter the blood, CO2 is very soluble and blown off, decreased PaO2 decreases SaO2 with normal PaCO2
any disease affecting the pulmonary intersitiaum
disease of alveoli: walls and lumen

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40
Q

what is the pulmonary intersitium

A

alveolar lining cells (types 1 and 2)

thin elastin-rich connective component contains capillary blood vessels

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41
Q

how does restrictive lung disease occur

A

alveolitis in early stage - injury with inflammatory cell infiltration
fibrosis in late stage - sub pleural + basal fibrosis

42
Q

what disease shows acute ILD

A

adult respiratory distress syndrome

43
Q

In restrictive lung disease wha tis the lung structure terminally replace with

A

dilated spaces surrounded by fibrous walls

44
Q

what happens as a result of restrictive lung disease

A

hypoxia

resp + cardiac failure

45
Q

what usually causes acute restrictive lung disease

A

virus

46
Q

what causes DPLD

A

fluid in alveolar air space due to pulmonary oedema

consolidation of alveolar space: lumen narrows (solid tissue in space)

47
Q

what causes cardiac pulmonary oedema

A

raided Pulmonary venous pressure, LVF

48
Q

what causes non-cardiac pulmonary oedema

A

normal pulmonary venous pressure with leaky pulmonary capillaries
sepsis/trauma = ARDS (shock lung), altitude sickness

49
Q

3 causes of lumen narrowing due to consolidation of alveolar space

A

infective pneumonia
infarction: PE, vasculitis
BOOP (COP): rheumatoid disease, drugs, crytogenic

50
Q

what causes infective pneumonia to result in consolidation. of alveolar space

A

viral: influenza, measles, chickenpox,
bacterial: pneumococcus, TB
fungal: HIV, pneumocystis
parasites: toxocara, filaria

51
Q

2 types of gransulomatous-alvolitis

A

extrinsic allergic alvolitis

sarcoidosis

52
Q

9 causes of alveolitis

A 
gransulomatous-alvolitis
drugs 
toxic gas (chlorine)
pulmonary fibrosis (rheumatoid/IPF)
Pneumoconiosis - dust disease
autoimmune
eosinophilic (type I &amp; type III hypersensitivity response)
carcinomatous
idiopathic (connective tissue disease/ IPF)
53
Q

what drugs cause alveolitis

A

amidorone
bleomycin, methotrexate
gold (not used)

54
Q

2 types of dust disease (pneumoconiosis)

A

fibrogenic: asbestos, silicosis

non-fibrogenic: siderosis (iron), senses (tin), baritones (barium)

55
Q

what autoimmune diseases cause alvolitis

A 
	SLE
	Polyarteritis
	Wegeners
	Churg-strauss
	Bechet’s
56
Q

4 causes of eosinophilic alveolitis

A

 Drugs: Nitrofurantoin
 Fungal : Asoergillosis
 Parasites: Toxocara, Ascaris, Filaria
 Autoimmune: Churg Strauss, Polyarteritis

57
Q

symptoms of DPLD

A 
o	Breathless on exertion
o	Cough but no wheeze
o	Finger clubbing
o	Inspiratory Lung crackles
o	Central cyanosis (if hypoxaemic)
o	Pulmonary fibrosis occurs as end stage response to chronic inflammation
58
Q

is peak flow high, low, normal for DPLD

A

normal

59
Q

what disease arise due to IDL

A

idiopathic interstitial pneumonia: IPF, acute interstitial pneumonia, NSIP
sarcoidosis
extrinsic allergic alvoleitis

60
Q

what ILD is finger clubbing a sign of

A

idiopathic pulmonary fibrosis (cryptogenic fibrosis alvolitis and usual interstitial pneumonia)

61
Q

what is pneumoconiosis

A

Lung disease caused by mineral dust exposure
• Asbestosis
• Coal workers lung
• silicosis

62
Q

what is connective tissue disease

A
  • Interstitial fibrosis (milder than IPF)
  • Pleural effusions
  • Rheumatoid nodules
63
Q

how is serum used to detect sarcoid

A

raised ACE and Ca

64
Q

what is an echocardiogram used to diagnose

A

heart failure

secondary pulmonary hypertension

65
Q

what would a HRCT of a patient with ILD show

A

inflammatory ground glass

66
Q

what would a HRCT of a patient with IPF show

A

fibrotic nodular component of alveolar infilatrates

67
Q

what are the 2 types of lung biopsy

A

trans bronchial

thoracoscopic: more reliable - more tissue, more invasive

68
Q

if there is a ground glass appearance shown on HRCT what treatment should be given

A

immunosuppressives treat reversible alveolitis

69
Q

what is the first line treatment t of ILD

A

Oral prednisolone (not inhaled)

70
Q

what is the second line treatment of ILD

A

oral azathioprine

71
Q

what is sarcoidosis

A

multiple-system granulonatous of unknown cause
non-caseating granuloma + abnormal antigen trigger CD4+ T cell response
Type IV hypersensitivity response to unknown antigen

72
Q

what is a granuloma

A

mass/nodule of chronically inflamed tissue formed by macrophages, insoluble

73
Q

what do scarred granulomas consist of

A

accumulation of epithelia cels, macrophages, lymphocytes

74
Q

what is sarcoid due to

A

interstitial lung disease

75
Q

what type of sensitivity reaction is sarcoid

A

Type IV

imbalance of immune system due to inhalation of unknown antigen

76
Q

how are sarcoid granulomas formed

A

alveolar macrophages, CD4+, CD8+ and B cells in lung parenchyma
macrophages produce TNFa, free radicals, IL-12 stimulates Th1 cells releasing IFNy which acts on marcophages

77
Q

what does persistent immune activation lead to

A

granuloma build up

tissue damage and fibrosis

78
Q

what causes sarcoid

A

exposure of genetically susceptible person to antigenic trigger

79
Q

give 3 examples of antigenic triggers that can cause sarcoid

A

infective: mycobacteria
geographic: pine pollen
occupation: beryllium

80
Q

is sarcoid more or less common in smokers

A

less common

81
Q

what people are at higher risk of sarocid

A 
Afro-carribeans
irish
scandinavials
rural populations
USA
young people (20-40)
family history
not common in japan
82
Q

what do people with sarcoid present with

A 
can be asymotomatic
weightless
non-productive cough
dyspnoea
chest discomfort
83
Q

what type of sarcoid is more common in Caucasians

A

acute sarcoid

84
Q

how is acute sarcoid characterised

A 
pulmonary infiltrates
erythema nodosum
bilateral hilar lymphadenopathy
arthiritis
anterior uveitus
parotitis
fever
85
Q

what is erythema nodosum

A

painful lumps in shins, purple/pink tendon nodules, lasts few days
chilblain-like lesions = lupus pernio

86
Q

what is bilateral hilar lymphadenopathy

A 
chunky lymph nodes on chest x-ray
dull ache in chest
malaise
mild fever
sweats at night
can resolve itself after a few months
87
Q

what is anterior uvitis

A

misted vision

more common in women and afro-carribean

88
Q

what is parotitis

A

bilateral uveitis + paratoid gland enlargement

89
Q

what is chronic sarcoidosis t

A

progressive ILD

increasing cough and dyspnoea

90
Q

how does chronic sarcoidosis present

A 
lung infiltrates (alvolitis) 
breathlessnes, purple skin rashes
skin infiltrations 
peripheral lymphadenopathy
hypercalcaemia
lung fibrosis 
resp failure
91
Q

what does a CXR and HRCT of lungs with a patient with sarcoid

A

peripheral nodular infiltrate
nodule appearance
can be asymetric

92
Q

what does a trans bronchial tissue biopsy show in a patient with sarcoid

A

non-caseating granuloma

infiltration of alveolar walls and interstitial space in leukocytes, T cells

93
Q

TB and sarcoid can look similar how determine if the disease is sarcoid

A

tuberculin test is negative

Mantoux test rules out TB

94
Q

what does a blood test of a patient with sarcoid show

A

Angiotensin converting enzyme (ACE) raided by 2 deviations, marker activity (not diagnostic)
raised calcium
increased inflammatory markers (raised CRP)
mild normochromic normoytic anaemia with raised ESR
increased lymphocytes

95
Q

how is acute sarcoid treated

A

can be self-limiting usually no treatment required, watchful waiting
if vital organ affected give steroids to alleviate symptoms

96
Q

if sarcoid is steroid resistant what should be given

A

immunosuppressive therapy

97
Q

treatment for chronic sarcoid

A

oral steroids - prednisolone
immunosuppression: methotrexate is first line, other options: azathioprine, anti-TNF also treats rheumatoid arthritis and used if everything else fails

98
Q

why are sarcoid patins chest x-rays and pulmonary function monitored for several years

A

it often relapses

99
Q

what is the prognosis of resp failure

A

more severe in certain groups : Black Americans 10% death rate
death due to resp failure
initial chest x-ray = good prognosis guide

100
Q

what is the most useful way to monitor a lung disease progression

A

lung volume, peak flow and gas transfer

RESPIRATORY (12 decks)

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