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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
if untreated what does IPF lead to
resp failure pulmonary hypertension cor pulmonale
26
is IPF restrictive or obstructive lung disease
restrictive reduced FEV1 and FVC with normal/raised FEV1/FVC ratio reduced lung volumes low gas transfer
27
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
IPF
28
what does a patient which IPF show on CT scan
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
29
what test can be carried out if unsure IPF after chest x-ray and HRCT scan
lung biopsy - trans bronchial or throacicscopic
30
describe the blood gasses of a patient with IPF
arterial hyperaemia due to alveolar-capillary block | ventilation-perfusion mismatch with normal/low PaCO2 due to hyperventialtion
31
what would a blood test of a patient with IPF show
anti-nuclear antibodies rheumatoid factors ESR + antibodies = elevated
32
there would be increased/decreased neutrophils and macrophages in bronchoalveolar lavage of a patient with IPF
increased
33
what is the treatment of IPF
best supportive care steroids/immunosuppresants does not reverse fibrosis but can slow progression transplant in young patients <60
34
what can be given in an attempt to disable IPF
prednisolone | azathioprine/cyclophosphamide added if no response
35
what new drugs are in trial to treat IPF
Prifenidone and Nintadanib side effects: diarrhoea, nausea, sun sensitivity very expensive
36
is IPF usually a disease of younger or older people
older >70
37
what is the prognosis of IPF
most patients progress into resp failure in first few years | 4 year survival from point of diagnosis
38
what is another name of diffuse parenchymal lung disease
restrictive thoracic disease -
39
what is DPLD
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
40
what is the pulmonary intersitium
alveolar lining cells (types 1 and 2) | thin elastin-rich connective component contains capillary blood vessels
41
how does restrictive lung disease occur
alveolitis in early stage - injury with inflammatory cell infiltration fibrosis in late stage - sub pleural + basal fibrosis
42
what disease shows acute ILD
adult respiratory distress syndrome
43
In restrictive lung disease wha tis the lung structure terminally replace with
dilated spaces surrounded by fibrous walls
44
what happens as a result of restrictive lung disease
hypoxia | resp + cardiac failure
45
what usually causes acute restrictive lung disease
virus
46
what causes DPLD
fluid in alveolar air space due to pulmonary oedema | consolidation of alveolar space: lumen narrows (solid tissue in space)
47
what causes cardiac pulmonary oedema
raided Pulmonary venous pressure, LVF
48
what causes non-cardiac pulmonary oedema
normal pulmonary venous pressure with leaky pulmonary capillaries sepsis/trauma = ARDS (shock lung), altitude sickness
49
3 causes of lumen narrowing due to consolidation of alveolar space
infective pneumonia infarction: PE, vasculitis BOOP (COP): rheumatoid disease, drugs, crytogenic
50
what causes infective pneumonia to result in consolidation. of alveolar space
viral: influenza, measles, chickenpox, bacterial: pneumococcus, TB fungal: HIV, pneumocystis parasites: toxocara, filaria
51
2 types of gransulomatous-alvolitis
extrinsic allergic alvolitis | sarcoidosis
52
9 causes of alveolitis
``` gransulomatous-alvolitis drugs toxic gas (chlorine) pulmonary fibrosis (rheumatoid/IPF) Pneumoconiosis - dust disease autoimmune eosinophilic (type I & type III hypersensitivity response) carcinomatous idiopathic (connective tissue disease/ IPF) ```
53
what drugs cause alveolitis
amidorone bleomycin, methotrexate gold (not used)
54
2 types of dust disease (pneumoconiosis)
fibrogenic: asbestos, silicosis | non-fibrogenic: siderosis (iron), senses (tin), baritones (barium)
55
what autoimmune diseases cause alvolitis
```  SLE  Polyarteritis  Wegeners  Churg-strauss  Bechet’s ```
56
4 causes of eosinophilic alveolitis
 Drugs: Nitrofurantoin  Fungal : Asoergillosis  Parasites: Toxocara, Ascaris, Filaria  Autoimmune: Churg Strauss, Polyarteritis
57
symptoms of DPLD
``` 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
is peak flow high, low, normal for DPLD
normal
59
what disease arise due to IDL
idiopathic interstitial pneumonia: IPF, acute interstitial pneumonia, NSIP sarcoidosis extrinsic allergic alvoleitis
60
what ILD is finger clubbing a sign of
idiopathic pulmonary fibrosis (cryptogenic fibrosis alvolitis and usual interstitial pneumonia)
61
what is pneumoconiosis
Lung disease caused by mineral dust exposure • Asbestosis • Coal workers lung • silicosis
62
what is connective tissue disease
* Interstitial fibrosis (milder than IPF) * Pleural effusions * Rheumatoid nodules
63
how is serum used to detect sarcoid
raised ACE and Ca
64
what is an echocardiogram used to diagnose
heart failure | secondary pulmonary hypertension
65
what would a HRCT of a patient with ILD show
inflammatory ground glass
66
what would a HRCT of a patient with IPF show
fibrotic nodular component of alveolar infilatrates
67
what are the 2 types of lung biopsy
trans bronchial | thoracoscopic: more reliable - more tissue, more invasive
68
if there is a ground glass appearance shown on HRCT what treatment should be given
immunosuppressives treat reversible alveolitis
69
what is the first line treatment t of ILD
Oral prednisolone (not inhaled)
70
what is the second line treatment of ILD
oral azathioprine
71
what is sarcoidosis
multiple-system granulonatous of unknown cause non-caseating granuloma + abnormal antigen trigger CD4+ T cell response Type IV hypersensitivity response to unknown antigen
72
what is a granuloma
mass/nodule of chronically inflamed tissue formed by macrophages, insoluble
73
what do scarred granulomas consist of
accumulation of epithelia cels, macrophages, lymphocytes
74
what is sarcoid due to
interstitial lung disease
75
what type of sensitivity reaction is sarcoid
Type IV | imbalance of immune system due to inhalation of unknown antigen
76
how are sarcoid granulomas formed
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
what does persistent immune activation lead to
granuloma build up | tissue damage and fibrosis
78
what causes sarcoid
exposure of genetically susceptible person to antigenic trigger
79
give 3 examples of antigenic triggers that can cause sarcoid
infective: mycobacteria geographic: pine pollen occupation: beryllium
80
is sarcoid more or less common in smokers
less common
81
what people are at higher risk of sarocid
``` Afro-carribeans irish scandinavials rural populations USA young people (20-40) family history not common in japan ```
82
what do people with sarcoid present with
``` can be asymotomatic weightless non-productive cough dyspnoea chest discomfort ```
83
what type of sarcoid is more common in Caucasians
acute sarcoid
84
how is acute sarcoid characterised
``` pulmonary infiltrates erythema nodosum bilateral hilar lymphadenopathy arthiritis anterior uveitus parotitis fever ```
85
what is erythema nodosum
painful lumps in shins, purple/pink tendon nodules, lasts few days chilblain-like lesions = lupus pernio
86
what is bilateral hilar lymphadenopathy
``` 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
what is anterior uvitis
misted vision | more common in women and afro-carribean
88
what is parotitis
bilateral uveitis + paratoid gland enlargement
89
what is chronic sarcoidosis t
progressive ILD | increasing cough and dyspnoea
90
how does chronic sarcoidosis present
``` lung infiltrates (alvolitis) breathlessnes, purple skin rashes skin infiltrations peripheral lymphadenopathy hypercalcaemia lung fibrosis resp failure ```
91
what does a CXR and HRCT of lungs with a patient with sarcoid
peripheral nodular infiltrate nodule appearance can be asymetric
92
what does a trans bronchial tissue biopsy show in a patient with sarcoid
non-caseating granuloma | infiltration of alveolar walls and interstitial space in leukocytes, T cells
93
TB and sarcoid can look similar how determine if the disease is sarcoid
tuberculin test is negative | Mantoux test rules out TB
94
what does a blood test of a patient with sarcoid show
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
how is acute sarcoid treated
can be self-limiting usually no treatment required, watchful waiting if vital organ affected give steroids to alleviate symptoms
96
if sarcoid is steroid resistant what should be given
immunosuppressive therapy
97
treatment for chronic sarcoid
oral steroids - prednisolone immunosuppression: methotrexate is first line, other options: azathioprine, anti-TNF also treats rheumatoid arthritis and used if everything else fails
98
why are sarcoid patins chest x-rays and pulmonary function monitored for several years
it often relapses
99
what is the prognosis of resp failure
more severe in certain groups : Black Americans 10% death rate death due to resp failure initial chest x-ray = good prognosis guide
100
what is the most useful way to monitor a lung disease progression
lung volume, peak flow and gas transfer

RESPIRATORY (12 decks)

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