Interstitial Lung disease Flashcards

1
Q

How is ILD characterized?

A

ILD is characterized by acute, subacute or chronic inflammatory infiltration of alveolar walls by cells, fluid and connective tissue

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2
Q

What is the result if the inflammation from ILD is left untreated?

A

If untreated, the inflammatory processes can progressively develop into irreversible pulmonary fibrosis

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3
Q

What is the occurrence of ILD in the united states?

A

81 out of every 100000 people have some form of ILD

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4
Q

What are the basic functions of the interstitium?

A

Support the lung structurally
Maintain fluid balance
Help repair and remodel the lungs

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5
Q

Describe the basic pathophysiologic pathway of ILD

A

Injury (inhaling shit)
Inflammation
Disordered repair of involved tissue
Pulmonary fibrosis
End stage lung disease

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6
Q

What happens to the alveoli in ILD?

A

The alveoli can be destroyed along with the adjacent pulmonary capillaries

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7
Q

What is the order of movement of ILD in regards to the tissues?

A

Moves from bronchi to alveolar walls to adjacent alveolar spaces

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8
Q

Describe what happens to the tissues as a result of ILD

A

Fibrotic thickening of the respiratory bronchioles alveolar ducts and alveoli

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9
Q

What can form in the lungs as a result of ILD?

A

Granulomas

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10
Q

Just what the fuck is a granuloma?

A

A granuloma is an aggregation of macrophages (along with other cells) that forms in response to chronic inflammation. This occurs when the immune system attempts to isolate foreign substances that it is otherwise unable to eliminate

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11
Q

What can form in the lungs specifically in patients with asbestosis?

A

Fibrocalcific pleural plaques

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12
Q

Describe how ILD changes the compliance of the lungs

A

ILD severely decreases compliance

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13
Q

What inorganic exposures can result in ILD?

A

Asbestos
Coal dust
Silica

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14
Q

Why is the size of asbestos fibers significant?

A

Asbestos fibers can be <1 micrometer in size which can allow them to be deposited very deeply in the respiratory tract, notably in the parenchyma

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15
Q

If you were looking at a tissue sample from someone who has asbestosis, what would it look like?

A

Asbestos fibers would appear within the thickened walls of the alveoli as brown or orange baton shaped structures

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16
Q

What can form in the lungs as a result of exposure to coal dust?

A

Pinpoint nodules called coal macules

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17
Q

Where do coal macules tend to be found?

A

Coal macules tend to be found in the first and second generation respiratory bronchioles and cause the adjacent alveoli to retract

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18
Q

What is the characteristic of complicated coal workers pneumoconiosis?

A

Coal workers pneumoconiosis is characterized by areas of fibrotic nodules greater than 1 cm in diameter
The nodules are composed of dense collagenous tissue with black pigmentation
Complicated CWP is characterized by nodules coalescing and forming large masses of fibrotic tissues

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19
Q

What is silicosis characterized by?

A

The formation of small rounded nodules scattered throughout the lungs

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20
Q

Describe hypersensitivity pneumonitis

A

Immune system detects antigens and produces antibodies that initiate an inflammatory response on future exposures
Pneumonitis develops after repeated/prolonged exposure to the antigen

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21
Q

What is complicated silicosis characterized by?

A

Same as complicated CWP
Nodules coalesce and form large masses of fibrous tissues, usually in the upper lobes and peripheral regions

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22
Q

What are examples of antibiotics that can result in ILD?

A

Bitrofurantoin - used for UTIs
Sulfazalazine - treating rheumatoid arthritis

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23
Q

What are examples of antiinflammatory agents that are linked to ILD?

A

Aspirin
Methotrexate

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24
Q

What are cardiovascular agents that are linked to ILD?

A

Amiodarone

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25
Q

T/F: Oxygen is linked to ILD

A

True, thats why we aggressively wean people

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26
Q

At what level of radiation do most patients have to worry about ILD?

A

> 6000 rad

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27
Q

Describe scleroderma

A

A relatively rare autoimmune disorder of the blood vessels and connective tissue

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28
Q

What does scleroderma do to the body?

A

Causes fibrous degeneration of the connective tissue of the skin, lungs and internal organs particularly in the esophagus, digestive tract, and kidney

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29
Q

Where are the effects of scleroderma most common found

A

The esophagus, digestive tract and kidneys
Can also affect the lungs

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30
Q

What does scleroderma of the lung appear as?

A

ILD and fibrosis

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31
Q

What does scleroderma do to the lungs

A

Causes significant scarring of the lung parenchyma

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32
Q

What are some pulmonary complications associated with scleroderma?

A

Diffuse interstitial fibrosis
Severe pulmonary hypertension
Pleural disease
Aspiration pneumonitis (secondary to esophageal involvement)

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33
Q

In what population is scleroderma most commonly seen?

A

Women, 30-50 yo

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34
Q

What is the link between rheumatoid arthritis and lung complications?

A

Medications used to relieve RA potentially raise risk for lung complications
RA can also be associated with fibrosis of the lungs and nodule formation

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35
Q

What mucous glands can Sjogrens syndrome effect that harm the lungs?

A

Tracheobronchial mucous glands
Glands atrophy, plugs form, atelectasis results, secondary infection to follow

35
Q

How does Sjogrens syndrom affect the lungs?

A

Pleurisy with/without effusion
Interstitial fibrosis
Lymphocyte infiltration of tracheal bronchial mucous glands resulting in mucous gland atrophy, mucous plug formation, atelectasis and secondary infection

36
Q

What occurs primarily in Sjogrens syndrome?

A

Lymphocytic infiltration of the salivary and lacrimal glands that results in dry mucous membranes in eyes and mouth

37
Q

What population is more affected by Sjogrens syndrome?

A

Women

37
Q

How does polymyositis/dermatomyositis affect the lungs?

A

Recurrent episodes of aspiration PNA due to weakening of pharynx muscles
Hypostatic PNA secondary to weakened diaphragm
Drug induced interstitial pneumonitis

38
Q

Describe polymyositis/dermatomyositis

A

Diffuse inflammatory disorder of striated muscles that weakens limbs, neck, and PHARYNX

39
Q

Describe lupus (systemic lupus erythematosus)

A

Autoimmune disease where the body attacks its own tissues
People with it are said to have a distinctive rash

40
Q

What are the pulmonary manifestations of lupus?

A

Pleurisy with or without effusion
Atelectasis
Diffuse infiltrates and pneumonitis
Diffuse ILD
Uremic pulmonary edema
Diaphragmatic dysfunction
Infections

41
Q

What forms during sarcoidosis?

A

Tubercles-noncaseating granulomas

42
Q

What increases in patients with sarcoidosis?

A

IgM, IgG, IgA

42
Q

What are the most commonly affected organ in patients with sarcoidosis?

A

Lungs

43
Q

Who is sarcoidosis most common in?

A

African americans
Women
Ages 10-40

44
Q

What does it mean if a disease is classified as idiopathic?

A

its an authoritative way of saying we dont know what causes this

45
Q

What does the imaging pattern “usual interstitial pneumonia” generally mean?

A

Idiopathic pulmonary fibrosis

46
Q

How might a CT appear in a patient with IPF (idiopathic pulmonary fibrosis)?

A

Honeycombing
Traction bronchiectasis (dilation of bronchi or bronchioles in areas of ILD)

47
Q

Tissue plugs are found in what disease process?

A

Cryptogenic organizing pneumonia
Aka bronchiolotos obliterans organizing PNA

48
Q

What happens in cryptogenic organizing PNA?

A

Connective tissue plugs in the small airways
Mononuclear cell infiltration of surrounding parenchyma

49
Q

Accumulation of lymphocytes in the lungs along with fibrosis is characteristic of what disease?

A

Lymphocytic interstitial PNA

50
Q

What happens in lymphocytic interstitial PNA

A

Diffuse pulmonary disorder characterized by fibrosis and accumulation of lymphocytes in the lungs

51
Q

What is commonly associated with lymphocytic interstitial PNA?

A

Lymphoma (cancer of the lymphatic system)

52
Q

What is required to diagnose Lymphocytic interstitial PNA?

A

Surgical lung biopsy

53
Q

The common pathology of inflammation and destruction of pulmonary vessels is characteristic of what group of disorders?

A

Pulmonary vasculitides

54
Q

What does Goodpastures syndrome cause in the kidneys?

A

Glomerulonephritis

55
Q

What is the pulmonary manifestation of granulomatosis with polyangiitis (wegeners granulomatosis)?

A

Numerous nodules in the lungs 1-9 cm in diameter and more commonly found in the upper lobes
Larger lesions that may cavitate

56
Q

What does Goodpasture’s syndrome cause in the lungs?

A

Recurrent episodes of pulmonary hemmorrhage
Pulmonary fibrosis

57
Q

What is the incidence of anti-GBM disease?

A

Less than 2 per million

57
Q

What else is Goodpasture’s syndrome called?

A

Anti GBM disease
Anti glomerular basement membrane disease

58
Q

What is the survival diagnosis for anti-GBM disease?

A

50% die from massive pulmonary hemorrhage
50% die from chronic renal failure

59
Q

What do patients suffering from idiopathic pulmonary hemosiderosis experience?

A

Recurrent episodes of pulmonary hemorrhage

60
Q

What happens in chronic eosinophilic PNA?

A

Alveoli and interstitium are infiltrated by eosinophils and macrophages

61
Q

How does chronic eosinophilic PNA present on a CXR?

A

Photographic negative ie lots of black spaces

62
Q

What are the main clinical manifestations of ILD?

A

Increased alveolar capillary membrane thickness
Excessive bronchial secretions

63
Q

What vital signs might be increased on a patient with ILD?

A

RR
HR
BP

64
Q

If you perform a chest assessment on a patient suspected of having ILD, what findings would indicate the presence of ILD?

A

Increased tactile and vocal fremitus
Dull percussion note
Bronchial breath sounds
Fine dry crackles (velcro)
Pleural rub
Whisper pectoriloquy

64
Q

During a physical examination, what might you observe in a patient with ILD

A

Cyanosis
Digital clubbing
Peripheral edema and venous distention
Enlarged and tender liver

65
Q

What would a PFT look like from a person with ILD?

A

Decreased capacities, particularly FVC
Normal or increased FEV1/FVC ratio
Decreased volumes

66
Q

What seemingly unrelated conditions may be indicative of ILD?

A

Dry mouth
Tooth or gum erosion
Abnormalities of the eyes or skin

67
Q

Why would the FEV1/FVC remain normal or even increase?

A

ILD increases the elastic recoil force of the lungs making it easier to expel air

68
Q

Which disease processes are exceptions when considering ILD and DLco?

A

Goodpasture’s syndrome
Idiopathic pulmonary hemosiderosis

68
Q

ILD has what effect on hemodynamic indices?

A

Increased pulmonary arterial pressure
Increased pulmonary vascular resistance

68
Q

What happens to the diffusion capacity in patients with ILD?

A

Diffusion capacity decreases as a result of increase AC membrane thickness

69
Q

What might you see on a CBC from a patient you suspect to have ILD?

A

Increased hematocrit
Increased hemoglobin

70
Q

A patient with ILD or suspected of ILD might have what findings on a CXR?

A

Bilateral reticulonodular pattern
Irregularly shaped opacities
Granulomas
Cavity formation
Honeycombing
Pleural effusion

71
Q

What the fuck does reticulonodular mean?

A

Reticular = net like
Node = small defined mass of tissue

72
Q

What might the CXR of an individual with asbestosis look like?

A

Cloudy or ground glass opacity
Might have diaphragmatic or pleural plaques

73
Q

How do sarcoid cysts appear on a CXR?

A

They look like a honeycomb pattern

74
Q

How can you test for ILD?

A

CXR (not adequate to diagnose)
High resolution CT scan
Bronchoscopy for BAL
Biopsy

75
Q

What is the difference in resolution between a normal CT scan and a high resolution CT scan?

A

Normal resolution of 1-2 mm
High resolution of 100-200 micrometers

76
Q

What is the gold standard for assessing ILD?

A

High Resolution CT scan

77
Q

What is the best way to care for individuals with ILD?

A

By supporting their physical condition, understanding medications, supporting effective nutrition and education surrounding movement patterns that will allow them to conserve energy and improve their quality of life

78
Q

T/F: Lung transplants are not a viable option for patients with ILD

A

False. They can be hard to come by but they are a good option for individuals with severe ILD

79
Q

What is the focus of palliative care?

A

On maintaining quality of life