Brainscape's Knowledge GenomeTM

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


See full index

Year 3 Respiratory > Interstitial lung disease > Flashcards

Interstitial lung disease Flashcards

1
Q

What is idiopathic pulmonary fibrosis?

A

Chronic, inflammatory condition of the lung resulting in fibrosis of alveoli and interstitium. Also known as idiopathic fibrosis alveolitis and cryptogenic fibrosing alveolitis.

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

What is the aetiology of idiopathic pulmonary fibrosis?

A

Cause is unknown. However, in a genetically pre-disposed host, recurrent injury to alveolar epithelium may result in secretion of cytokines and growth factors which cause fibroblast activation, recruitment, proliferation, differentiation into myofibroblasts, increased collaged synthesis and deposition.

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

What are the risk factors of IPF? (x5)

A

Smoking (in 75%), occupational exposure to metal or wood dust, GORD (chronic microaspiration), diabetes and infection (HepC, adenovirus, cytomegalovirus, EBV).

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

What is the pathophysiology of IPF?

A

Initial stages of disease involved pro-inflammatory and pro-fibrotic response involving influx of macrophages, fibroblasts and basement membrane. The main pathology involves enhanced and dysregulated activity of fibroblasts and myofibroblasts, resulting in alveolar destruction, fibrosis of interstitial space, and architectural distortion of lung parenchyma.

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

Taxonomy of idiopathic pulmonary fibrosis?

A

Interstitial idiopathic pneumonia is an umbrella term for interstitial lung diseases of unknown aetiologies including IPF (identified histologically as usual interstitial pneumonia (UIP), desquamative interstitial pneumonia (DIP), non-specific interstitial pneumonia (NSIP), and others. They can all be differentiated by their histological presentations.

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

What is the epidemiology of IPF: Prevalence? Gender? Age?

A

Rare. Prevalence is 6 in 100 000 in UK. Male:female 2:1. Mean age is 67.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

What are the symptoms of IPF? (x3)

A
  • Gradual onset of dyspnoea on exertion
  • Dry irritating cough, no wheeze
  • Fatigue and weight loss
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

What are the signs of IPF? (x3)

A
  • Finger clubbing
  • Bibasal fine late inspiratory crepitations
  • Signs of right heart failure in advanced stages (right ventricle has to overcome resistance of pulmonary circuit) – right ventricular heave, raised JVP, peripheral oedema.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

!!! How does IPF present on a chest x-ray?

A
  • Usually normal at presentation.
  • Early disease may feature small lung fields (lungs don’t look as big) and ‘ground glass’ shadowing (increased attenuation on X-ray with preserved lung markings)
  • Later, there is reticulonodular shadowing (especially at bases), signs of cor pulmonale, and honeycombing.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

What is reticulonodular shadowing?

A

A type of opacification containing nodule opacities (you can literally see nodules), and reticular opacities (network of fine lines representing interstitial changes).

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

What is cor pulmonale?

A

Enlarged right ventricle associated with right heart failure and pulmonary hypertension.

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

What is honeycombing?

A

Sign of widespread fibrosis: small cystic spaces with irregularly thickened walls composed of fibrous tissue. Cystic spaces arise from dilated and thickened alveoli.

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

What other investigations are there for IPF? (x6 (x2, x3, x4, x1, x1))

A
  • BLOODS: ABG shows low PO2 on exercise, normal PCO2 which rises in late disease. One-third have rheumatoid factor or ANA.
  • HIGH RESOLUTION CT: reticular densities, honeycombing and traction bronchiectasis PULMONARY FUNCTION TESTS: RESTRICTIVE picture (low FEV1, low FVC with preserved or increased ratio), decreased lung volumes, decreased lung compliance and decreased TLCO (diffusing capacity of the lungs for carbon monoxide)
  • BRONCHOALVEOLAR LAVAGE: to exclude infections/malignancy
  • LUNG BIOPSY: gold standard for diagnosis and shows USUAL INTERSTITIAL PNEUMONIA (interstitial fibrosis and honeycomb lung)
  • ECHOCARDIOGRAPHY: enlarged right ventricle
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

What is sarcoidosis?

A

Multisystem inflammatory disorder of unknown cause characterised by formation of noncaseating granulomas, affecting the lung in over 90% of cases.

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

What is the aetiology of sarcoidosis?

A
  • Unclear
  • There may be genetic predisposition
  • Mechanism suggests that the disease is an INFECTIVE disease e.g., there is accumulation of oligoclonal T cells at sites of granuloma formation, and features resemble antigen-induced disorders such as chronic beryllium disease, and hypersensitivity pneumonitis.
  • Associated with viruses, atypical mycobacterium, and Propionibacterium acnes
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

What are the risk factors of sarcoidosis? (x3)

A

Agricultural exposures, insecticides, microbial bioaerosols (work environments with mould/damp exposure).

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

What is a protective factor in sarcoidosis?

A

Smoking

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

What is the epidemiology of sarcoidosis: Age? Ethnicity? Gender? UK Prevalence?

A

Presenting between 20 and 40 years old, Africans, and females. Prevalence in UK is 16 in 100 000 and highest in Irish women.

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

What is the pathophysiology of sarcoidosis?

A

We do not know what the initial process step is. An APC displays an antigen, which cross-links with CD4+ Th1 cells, leading to macrophage activation, amplification of the immune response, and non-caseating granuloma formation in a number of organs. Mainly IL-1, IL-2, as well as IL-15, IL-18, gamma-IFN, MCP1 promote and maintain the granulomas. In the lungs, this leads to fibrosis of the lung parenchyma – hence sarcoidosis is an interstitial lung disease.

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

What is the disease progression of sarcoidosis?

A

Majority have a benign course with/without a period of treatment; significant minority have progressive pulmonary fibrosis.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

What are the signs and symptoms of sarcoidosis?

A
  • GENERAL: fever, malaise, weight loss, bilateral parotid swelling, LYMPHADENOPATHY, hepatosplenomegaly
  • LUNGS: breathlessness, unproductive cough, minimal clinical signs: fine inspiratory crepitations
  • MUSCULOSKELETAL: bone cysts, polyarthralgia, myopathy
  • EYES: keratoconjunctivitis sicca (dryness of conjunctiva), uveitis, papilledema (optic nerve swelling)
  • SKIN: lupus pernio (red-blue infiltrations of nose, cheek, ears, terminal phalanges; see photo), erythema nodosum (red patches from inflammation of subcutaneous fat), maculopapular eruptions (flat, small, confluent lumps)
  • NEUROLOGICAL: lymphocytic meningitis, space-occupying lesions, pituitary infiltration, cerebellar ataxia, cranial nerve palsies, peripheral neuropathy
  • HEART: arrythmia, BBB, pericarditis, cardiomyopathy, congestive heart failure
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

How does sarcoidosis spread?

A

Sarcoid granulomas tend to follow the lymphatic pathways. In the lungs, this means that it tracks down interlobular septa. Knowledge of this can help us identify sarcoidosis in radiology.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

What are the blood investigations for sarcoidosis? (x8)

A
  • High serum ACE
  • Hypercalcaemia from activated macrophages
  • High ESR
  • WCC may be decreased because of lymphocyte sequestration in the lungs
  • Anaemia secondary to bone marrow/spleen involvement
  • Polyclonal hyperglobulinaemia
  • Raised alkaline phosphatase and GGT
  • Deranged U&Es with renal involvement
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

How may sarcoidosis present on CXR?

A

Bilateral hilar lymphadenopathy and pulmonary infiltration with/without fibrosis.

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

How is sarcoidosis staged?

A
  1. **Staging done by CXR. Stage 1: bilateral hilar lymphadenopathy
  2. Stage 2: bilateral hilar lymphadenopathy and parenchymal infiltration
  3. Stage 3: parenchymal infiltration and NO hilar lymphadenopathy
  4. Stage 4: fibrosis and honeycombing
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
26
Q

How does sarcoidosis staging relate to prognosis?

A

Stage 1 is associated with spontaneous resolution. Stage 4 is associated with poor prognosis and permanent organ damage.

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

What is the problem with CXR in sarcoidosis? What can we do to confirm diagnosis? Especially in what circumstance?

A

Cannot differentiate between inflammation, fibrosis and inactive disease, so histology is needed to make a confident sarcoidosis diagnosis, particularly if immunosuppressive treatment is being considered.

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

What are the other investigations for sarcoidosis? (x6)

A
  • High-resolution CT: ground-glass opacity and lymphadenopathy. Cystic architectural distortion (with honeycombing) seen in severe disease
  • 67 Gallium scan: panda sign (lacrimal and parotid gland uptake; see photo), lambda sign (hilar and paratracheal lymphadenopathy uptake; see photo)
  • Pulmonary function tests: low FEV1, FVC and gas transfer – RESTRICTIVE picture
  • Bronchoscopy and bronchoalveolar lavage: raised lymphocytes and high CD4:CD8 ratio
  • Transbronchial lung biopsy
  • FDG-PET scan (contrast scan): activity of PET correlates with granulomatous inflammation.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
29
Q

What does transbronchial lung biopsy show in sarcoidosis? (x3)

A

Non-caseating granulomas composed of epithelioid cells (activated macrophages), multinucleate Langhans cells (found in granulomas; they are fused macrophages) and mononuclear cells.

30
Q

Why is it important to measure disease activity in sarcoidosis?

A

It can sometimes be inactive, which means that treatment would be unnecessary. We can just monitor the patient instead.

31
Q

What is extrinsic allergic alveolitis?

A

AKA hypersensitivity pneuomitis. Interstitial inflammatory disease of the distal gas-exchanging parts of the lung caused by inhalation of organic dusts.

32
Q

What is the aetiology of extrinsic allergic alveolitis? Common agents?

A
  • Inhalation of antigenic organic dusts containing microbes or animal proteins induce a hypersensitivity response in susceptible individuals.
  • The two most commonly reported agents are bacteria thermophilic actinomycetes, responsible for Farmer’s lung and Mushroom Picker’s lung, and animal proteins e.g., avian proteins responsible for Pigeon Breeder’s lung, Bird Fancier’s lung, and Budgerigar Fancier’s disease
33
Q

What is the pathophysiology of extrinsic allergic alveolitis?

A

A combination of Type III antigen-antibody complex hypersensitivity reaction (of IgE) and a type IV granulomatous lymphocytic inflammation in ALVEOLI and BRONCHIOLES. The cellular infiltrate consists primarily of lymphocytes, plasma cells, and neutrophils. In addition, there are non-caseating granulomas and activated foamy macrophages. Later stages are associated with interstitial fibrosis.

34
Q

What are the risk factors for extrinsic allergic alveolitis? (x3)

A

Smoking, occupation (metal worker, farmer, painter), immunosuppressants.

35
Q

What is the epidemiology of extrinsic allergic alveolitis: Occupational lung diseases? Occupation? Where?

A

2% of occupational lung disease, 50% affect farm workers, marked geographical variation reflecting occupational causes.

36
Q

How is EAA categorised? (x3)

A

Acute (within hours), sub-acute (weeks-months), and chronic (develops months/years after exposure).

37
Q

What are the symptoms of acute versus chronic extrinsic allergic alveolitis?

A
  • ACUTE: Reversible episodes of dry cough, dyspnoea, malaise, fever, myalgia. Wheeze and productive cough may develop following repeat high-level exposure.
  • CHRONIC: Gradual onset breathlessness, cough, decreased exercise tolerance, and weight loss. Note that fever and malaise are absent.
38
Q

What are the signs of acute versus chronic extrinsic allergic alveolitis?

A

• ACUTE: Rapid shallow breathing, pyrexia, inspiratory crepitations. • CHRONIC: Fine inspiratory crepitations, finger clubbing (rare).

39
Q

How does extrinsic allergic alveolitis present on CXR?

A

• Often normal in acute episodes. • May show ground glass appearance. • Patchy, nodular infiltrates in acute and sub-acute – especially in middle and lower zones. • In chronic cases, fibrosis is prominent in the upper zones.

40
Q

What are the other investigations for extrinsic allergic alveolitis? (x5)

A
  • BLOODS: neutrophilia, lymphopenia, and reduced PO2 and PCO2 in ABG
  • SEROLOGY: precipitating IgG to fungal or avian antigens in serum but NOT diagnostic
  • CT THORAX: patchy ground glass shadowing and nodules
  • PULMONARY FUNCTION TESTS: restrictive picture – low FEV1, FVC and TLCO
  • BRONCHOALVEOLAR LAVAGE: increased cellularity with high CD*+ suppressor T cells.
41
Q

What is pneumoconiosis?

A

Fibrosing interstitial lung disease caused by chronic inhalation of mineral dusts.

42
Q

What are the types of pneumoconiosis? (x4)

A
  • Silicosis
  • Coal worker’s pneumoconiosis (black lung disease)
  • Chronic beryllium disease
  • Asbestosis from prolonged exposure to asbestos
  • There are many others but typically asymptomatic and benign despite CXR changes
43
Q

What is the aetiology of pneumoconiosis?

A

Caused by inhalation of particles of coal dust, silica or asbestos leading to interstitial fibrosis.

44
Q

What is the aetiology of silicosis?

A

Silica is silicon combined with oxygen (SiO2). When silica is heated, it is transformed into tridymite and cristobalite which are fibrogenic.

45
Q

What are the risk factors of pneumoconiosis? (x3)

A

Occupational exposure (coal mining, quarrying, iron and steel foundries, stone cutting, sand blasting, insulation industry, plumbers, ship builders). Susceptibility also linked to smoking and TB.

46
Q

What is the pathophysiology of silicosis and coal worker’s pneumoconiosis?

A
  • There are large nodules in the lung consisting of dust particles surrounded by layers of collagen and dying macrophages. There are four mechanisms:
  • Direct cytotoxicity by particles accessing alveoli
  • Particle ingestion by macrophages results in activation and excessive free radical production causing lipid peroxidation, cell injury and macrophage cytolysis
  • Proinflammatory cytokines and growth factors from macrophages and epithelial cells stimulate fibroblast proliferation
  • Pateints also have polyclonal hypergammaglobulinemia (antibodies), though role is not clear.
47
Q

What is the pathophysiology of chronic beryllium disease?

A

Unlike silica and coal, exposure does not link to severity of disease. T cells bind to beryllium in the lungs and become sensitised. Subsequent exposures lead to CD4+ T cell and macrophage response leading to non-caseating granuloma formation, macrophage lysis and fibrosis.

48
Q

What is the epidemiology of pneumoconiosis: Where?

A

Incidence growing in developing countries.

49
Q

What are the symptoms of pneumoconiosis? (x3 +1 point)

A

• Often asymptomatic in silica and coal pneumoconiosis and picked up on routine CXR • Insidious onset of SOB • Dry cough • Black sputum (melanoptysis) in coal worker’s pneumoconiosis

50
Q

What are the signs of pneumoconiosis?

A
  • Decreased breath sounds, signs of pleural effusion or right heart failure (cor pulmonale).
  • Wheezing/crackling on auscultation in beryllium disease patients.
  • Advanced disease associated with areas of dullness on percussion, clubbing, cyanosis.
51
Q

Why is right heart failure associated with interstitial fibrosis?

A

Fibrosis increases pulmonary vascular resistance which leads to pulmonary hypertension.

52
Q

How does pneumoconiosis present on CXR?

A
  • SIMPLE: micronodular mottling is present
  • COMPLICATED: nodular opacities in the upper lobes, micronodular shadowing, eggshell calcification of hilar lymph nodes (characteristic of silicosis; see photo)
53
Q

What are the other investigations for pneumoconiosis? (x3)

A
  • CT: fibrotic changes.
  • Bronchoscopy: visualise changes and allows for bronchoalveolar lavage (which will show granulomas in beryllium patients)
  • Lung function tests: restrictive ventilatory defect and impaired gas diffusion
54
Q

What is the advantage of CT scanning in all interstitial fibrotic diseases?

A

It can identify fibrotic changes earlier than CXR.

55
Q

What is asbestosis?

A

Diffuse interstitial fibrosis of lungs as a result of asbestos fibre exposure.

56
Q

What is the aetiology of asbestosis and asbestos-related pleural changes?

A

There are two main types of asbestos fibre: white fibres (called Chrysotile), and blue fibres (Crocidolite). The latter is more toxic. Amount inhaled is related to the risk of developing plaques or the disease.

57
Q

What is the pathophysiology of asbestos-related plaque formation? (x4 points)

A
  • Asbestos fibres deposit at alveolar duct bifurcations and cause alveolar macrophage alveolitis. These activated macrophages release cytokines which initiate fibrosis.
  • Initially, this disease process begins in the lower lobes and may progress to honeycombing.
  • Histologically, areas of pathology are called asbestos bodies. Bodies consisting of fibres coated in iron and haemosiderin are seen in some forms of asbestosis.
  • Often, pleural plaques contain few asbestos fibres because they white fibres (which are most common) are broken down and cleared form the lung via lymphatics.
58
Q

What is the epidemiology of asbestos-related disease: Where?

A

Increasing in developing countries.

59
Q

What are the risk factors of asbestosis?

A

Occupation exposure (shipyard, construction, mechanic) and smoker.

60
Q

What are the signs and symptoms of asbestosis? (x4)

A
  • Dyspnoea on exertion
  • Cough; dry and non-productive
  • End-inspiratory crepitations, especially at lung bases
  • Clubbing in advanced disease
61
Q

What chest pain is associated with asbestos-related disease?

A

Pleuritic chest pain as a result of acute asbestos pleurisy (inflammation of pleura).

62
Q

How does asbestosis present on CXR? (x2)

A
  • Bilateral lower zone reticulonodular shadowing (interstitial fibrosis) and pleural plaques, visible as white lines when calcified, often most obvious on the diaphragmatic pleura or as ‘holly leaf’ patterns (irregular nodular edges).
  • Bilateral pleural thickening uncommon but highly specific
63
Q

What are the other investigations for asbestosis? (x4)

A
  • Pulmonary function tests: Restrictive
  • CT chest: lower zone linear interstitial fibrosis and pleural thickening
  • Lung biopsy: asbestos bodies and interstitial fibrosis
  • Bronchial lavage: presence of asbestos bodies
64
Q

What is mesothelioma?

A

Epithelial neoplasm arising from mesothelium of the lung pleura and related to asbestos exposure.

65
Q

What is the aetiology/risk factors of mesothelioma? (x4)

A

Asbestos-exposure, genetic predisposition, radiotherapy and the simian virus 40 (SV-40).

66
Q

What is the pathophysiology of mesothelioma?

A

Chronic inflammation and oxidative stress from asbestos leads to activation of macrophages which culminates in DNA damage and eventual malignant transformation of the mesothelium of the visceral pleura. These develop into MALIGNANT mesothelial plaques because asbestos fibres are carried to the pleural surface.

67
Q

What are the main complications of mesothelioma? (x2)

A

Mesothelioma is associated with pleural effusion and trapped lung (cannot fully expand from replacement of hemithorax with mesothelioma).

68
Q

What are the signs and symptoms of mesothelioma? (x6)

A
  • SOB due to pleural effusion or trapped lung
  • Diminished breath sounds from pleural effusion or trapped lung
  • Dullness to percussion from pleural effusion
  • Chest pain when mesothelioma invades chest wall
  • Dry, non-productive cough
  • Constitutional symptoms
69
Q

How does mesothelioma present on CXR? (x3)

A
  • Unilateral pleural effusion
  • Irregular pleural thickening
  • Lower zone interstitial fibrosis
70
Q

What are the other investigations for mesothelioma? (x5)

A
  • CT with contrast: pleural thickening, pericardial effusions, mediastinal lymph nodes, chest wall invasion
  • Cytology (thoracentesis or pleural biopsy): cytological confirmation of malignancy can be obtained in pleural biopsy. Thoracentesis may show malignant cells in pleural fluid but low sensitivity
  • Video-assisted thoracoscopic surgery (VATS): to evaluate pleural lining; pleural thickening or discrete plaques
  • MRI: assess tumour extension
  • PET scan: evaluates location and tumour extension
71
Q

What drugs cause pulmonary fibrosis? (x6)

A

Amiodarone, bleomycin, bulsulfan, nitrofurantoin, methotrexate and sulfasalazine are drugs that can cause pulmonary fibrosis with long-term use.

Year 3 Respiratory (14 decks)

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