Fibrotic Lung Disease Flashcards

1
Q

What is sarcoidosis?

A

abnormal collections of inflammatory cells such as epithelioid cells, macrophages and T cells forming clumps in the lungs, skin and lymph nodes

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2
Q

In which ethnic demographic is sarcoidosis most common?

A

Afro-Caribbean

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3
Q

Is sarcoidosis more common in males or females?

A

Females

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4
Q

Give 3 constitutional clinical features of sarcoidosis:

A
  1. Fever
  2. Weight loss
  3. Fatigue
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5
Q

Give 3 renal clinical features of sarcoidosis:

A

1) granulomatous tubulointerstitial nephritis
2) hypercalcaemia
3) stone formation

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6
Q

Give 3 respiratory clinical features of sarcoidosis:

A

1) cough
2) dyspnoea
3) wheeze

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7
Q

Give 2 ocular clinical features of sarcoidosis:

A

1) anterior uveitis
2) keratoconjunctivitis sicca

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8
Q

What is keratoconjunctivitis sicca?

A

Dry eye syndrome

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9
Q

What is uveitis?

A

Inflammation of the uveal tract (iris, ciliary body, choroid)

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10
Q

Give 3 immunopathological features of sarcoidosis:

A

1) negative Mantoux test
2) overall lymphopenia
3) infiltration of the alveolar walls and interstitial spaces

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11
Q

Give two reticuloendothelial features of sarcoidosis:

A

1) splenomegaly
2) lymphadenopathy

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12
Q

Give two cutaneous features of sarcoidosis:

A

1) erythema nodosum
2) lupus pernio

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13
Q

What is erythema nodosum?

A

Tender red nodules caused by inflammation of fat cells usually found on the shins

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14
Q

What is lupus pernio?

A

raised discoloration of nose/ear/cheek/chin which resembles frostbite

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15
Q

Give six neurological features of sarcoidosis:

A

1) cognitive dysfunction
2) headache
3) cranial nerve palsies
4) mononeuritis multiplex
5) peripheral neuropathy
6) seizures

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16
Q

What is mononeuritis multiplex?

A

nerve damage in 2 or more nerves in separate parts of the body

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17
Q

Give 4 cardiac features of sarcoidosis:

A

1) arrhythmias
2) heart block
3) cardiomyopathy
4) sudden death

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18
Q

Describe the CXR findings of stage 1 pulmonary sarcoidosis:

A

bilateral hilar lymphadenopathy

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19
Q

Describe the CXR findings of stage 2 pulmonary sarcoidosis:

A

pulmonary infiltrates with bilateral hilar lymphadenopathy

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20
Q

Describe the CXR findings of stage 3 pulmonary sarcoidosis:

A

pulmonary infiltrates without bilateral hilar lymphadenopathy

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21
Q

Describe the CXR findings of stage 4 pulmonary sarcoidosis:

A

Fibrosis

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22
Q

True or false: the chest will always sound clear in sarcoidosis regardless of the extent of infiltration

A

true

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23
Q

What enzyme is raised in sarcoidosis?

A

angiotensin converting enzyme

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24
Q

Does interstitial/ fibrotic lung disease cause an obstructive or restrictive lung defect?

A

Restrictive

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25
Q

Give 6 investigations used to diagnose and manage sarcoidosis:

A

1) CXR (for staging)
2) HRCT (for assessment of parenchymal involvement)
3) U+Es for hypercalcaemia
4) serum ACE levels
5) lung function tests (will show restrictive defects)
6) bronchoscopy and transbronchial biopsy

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26
Q

Why is hypercalcaemia associated with sarcoidosis?

A

activated macrophages in the lung and lymph nodes are able to hydroxylate vitamin D directly independent of parathyroid levels leading to an increased intestinal absorption of dietary calcium

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27
Q

How is severe sarcoidosis managed? (2)

A

1) prednisolone
2) in some cases, immunosuppressants such as methotrexate, azathioprine and hydroxychloroquine

28
Q

What are idiopathic interstitial pneumonias?

A

interstitial lung diseases with no known cause

29
Q

What is the most common form of idiopathic interstitial pneumonias?

A

idiopathic pulmonary fibrosis

30
Q

True or false: idiopathic pulmonary fibrosis is fatal

A

True

31
Q

Give 3 HRCT findings associated with idiopathic pulmonary fibrosis:

A

1) progressive scarring seen as honey combing (mainly in the subpleural areas)
2) reticulation (linear opacities)
3) bronchiectasis

32
Q

Describe the pathophysiology of idiopathic pulmonary fibrosis:

A

repetitive injury to the alveolar epithelium caused by an unknown environmental stimuli leads to the activation of repair pathways which become uncontrolled, leading the overproduction of fibroblasts and increased deposition of increased extracellular matrix in the interstitial

33
Q

Give 4 clinical features associated with idiopathic pulmonary fibrosis:

A

1) cough
2) progressive dyspnoea
3) finger clubbing
4) pulmonary hypertension

34
Q

Give 4 investigations used to diagnose and manage idiopathic pulmonary fibrosis:

A

1) CXR
2) HRCT
3) ANA and Rheumatoid Factor blood tests (to rule out autoimmune rheumatic disease)
4) Respiratory function test

35
Q

What is the mean survival time for a patient with idiopathic pulmonary fibrosis?

A

2-5 years

36
Q

Give 2 drugs that can be used to help manage idiopathic pulmonary fibrosis:

A

1) pirfenidone
2) nintedanib

37
Q

Describe how pirfenidone can help manage idiopathic pulmonary fibrosis:

A

it acts as an antifibrotic agent that has been shown to slow the rate of forced vital capacity (FVC) decline

38
Q

Describe how nintedanib can help manage idiopathic pulmonary fibrosis:

A

it is an intracellular inhibitor of tyrosine kinase, preventing activation of scarring repair pathways

39
Q

What is desquamative interstitial pneumonia? (5)

A

1) Presents with insidious onset of dyspnoea and dry cough over weeks/months (+/- clubbing of digits)
2) Smoking is VERY common
3) Often in 4-5th decade of life
4) Excellent response to Steroids and Smoking Cessation
5) It is caused by the accumulation of pigmented macrophages within alveolar spaces

40
Q

What is respiratory bronchiolitis interstitial lung disease?

A

where the lumen of respiratory bronchioles are filled with pigmented macrophages, appearing as centrilobular nodules on imaging

41
Q

What is acute interstitial pneumonia?

A

sudden dyspnoea and progressive respiratory failure due to diffuse alveolar damage with no known cause

42
Q

What is cryptogenic organising pneumonia?

A

Buds of connective tissue known as Masson bodies form in alveoli and alveolar ducts causing influenza-like symptoms with cough and dyspnoea

43
Q

What is lymphoid interstitial pneumonia?

A

where lung interstitial is infiltrated by lymphocytes, macrophages and plasma cells often seen in HIV, presenting with insidious dyspnoea, dry cough and systemic upset

44
Q

What is hypersensitivity pneumonitis?

A

inflammation of the lung parenchyma caused by antigen exposure

45
Q

Give 7 types of hypersensitivity pneumonitis:

A

1) Famer’s lung
2) Bird fancier’s lung
3) Malt worker’s lung
4) Humidifier fever
5) Mushroom worker’s lung
6) Cheese worker’s lung
7) Winemaker’s lung

46
Q

What allergen causes Farmer’s lung?

A

inhaling thermophilic actinomycetes (a group of bacteria found on decaying vegetations e.g. mouldy hay)

47
Q

What allergen causes Bird fancier’s lung?

A

proteins on feathers and faeces of birds

48
Q

What allergen causes Malt worker’s lung?

A

causes Malt worker’s lung?
inhaling Aspergillus clavatus antigens found on germinating bale

49
Q

What allergen causes Humidifier fever?

A

inhaling a variety of bacterium or amoeba from contaminated humidifying systems

50
Q

What allergen causes Mushroom worker’s lung?

A

inhaling thermophilic actinomycetes found on mouldy mushroom compost

51
Q

What allergen causes Cheese washer’s lung?

A

inhaling Penicillium casei and Aspergillus clavatus (associated with mouldy cheese)

52
Q

What allergen causes Wine maker’s lung?

A

inhaling Botrytis, a type of necrotrophic fungus associated with mould on grapes

53
Q

How does allergy lead to hypersensitivity pneumonitis?

A

cellular immunity and deposition of immune complexes results in chronic inflammatory infiltrates, interstitial granulomas and interstitial fibrosis

54
Q

Give 6 clinical features of hypersensitivity pneumonitis:

A

1) fever
2) weight loss
3) malaise
4) clubbing
5) cough
6) dyspnoea

55
Q

Give two auscultation findings associated with hypersensitivity pneumonitis:

A

1) inspiratory squeaks (due to bronchiolitis)
2) bilateral fine crackles

56
Q

Give 3 investigations used to diagnose and manage hypersensitivity pneumonitis:

A

1) CXR (diffuse nodules and increased reticular shadowing)
2) HRCT (shows nodules and honey comb changes)
3) antibody test (to test for particular allergens

57
Q

How is hypersensitivity pneumonitis treated? (2)

A

1) avoidance of inciting allergen
2) prednisolone

58
Q

Name 6 rare interstitial lung disease:

A

1) Langerhans cell histiocytosis
2) Pulmonary lymphangioleiomyomatosis (smooth muscle infiltration)
3) Pulmonary alveolar proteinosis (lipoproteins accumulation in alveoli)
4) small-vessel vasculitides
5) Anti-glomerular basement membrane disease
6) diffuse alveolar haemorrhage

59
Q

Give 3 examples of small-vessel vasculitides:

A

1) Granulomatosis with polyangiitis
2) Microscopic polyangiitis
3) Eosinophilic granulomatosis with polyangiitis

60
Q

What is the triad of anti-glomerular basement membrane disease?

A

1) pulmonary haemorrhage
2) glomerulonephritis
3) antibodies against the basement membrane

61
Q

Give 4 pulmonary manifestations of rheumatoid disease/ arthritis:

A

1) rheumatoid nodules
2) obliterative bronchiolitis (narrowing bronchioles until they close)
3) cricoarytenoidal arthritis (inflammation of larynx joint)
4) Caplan’s syndrome

62
Q

Give 3 clinical presentations associated with cricoarytenoidal arthritis:

A

1) dyspnoea
2) stridor
3) hoarseness

63
Q

What is Caplan’s syndrome?

A

Rheumatoid arthritis with silicosis

64
Q

What is the most common allergen associated with pulmonary infiltration with eosinophilia?

A

Aspergillus fumigatus

65
Q

Give 5 manifestations of Aspergillus fumigatus in the lung:

A

1) fibrosis
2) mucous plugs
3) lung collapse
4) aspergillomas (fungus ball often formed in old TB capsule)
5) invasive aspergillosis (lung is overwhelmed with fungus)

66
Q

What is the name given to the condition where irradiation of the lung during radiotherapy?

A

radiation pneumonitis