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Respiratory > Interstitial Lung Diseases > Flashcards

Interstitial Lung Diseases Flashcards

1
Q

What is another name for interstitial lung disease?

A

Diffuse parenchymal lung disease

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2
Q

What is the interstitium?

A

The tissue and space around the air sacs of the lungs

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3
Q

What is the parenchyma?

A

Portion of the lung involved in gas transfer—the alveoli, alveolar ducts and respiratory bronchioles

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4
Q

What is interstitial lung disease?

A

Disease of the air-blood barrier

A term for a group of conditions that primarily affect the lung parenchyma in a diffuse manner

They feature chronic inflammation and progressive interstitial fibrosis

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5
Q

What part of the lungs do interstitial lung diseases affect?

A

Interstitium: tissue + space around the air sacs of the lungs

Alveoli
Bronchioles
Blood vessels

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6
Q

What are the clinical features of interstitial lung diseases?

A

Dyspnoea on exertion

Non-productive paroxysmal cough (sudden attacks)

Abnormal breath sounds

Clubbing

Fine end inspiratory crepitations

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7
Q

What is the pathogenesis of interstitial lung diseases?

A

Bronchioles + alveoli are damaged

Fibrosis occurs between alveoli, a band of fibrous tissue within the lung parenchyma develops

This causes decreased expansion of the lungs
AND
impairs gas exchange

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8
Q

What are the 5 major groups of interstitial lung disease?

A
  1. Associated with systemic diseases
  2. Caused by environmental triggers
  3. Granulomatous disease
  4. Idiopathic
  5. Other
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9
Q

Which systemic diseases can cause interstitial lung disease?

A

Rheumatoid arthritis, ank spond

Vasculitis

SLE

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10
Q

Which environmental triggers can cause interstitial lung disease?

A

Drugs
Fungal
Dust

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11
Q

Name some granulomatous diseases that cause interstitial lung disease?

A

Sarcoidosis

Wegener’s

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12
Q

Name some idiopathic causes of interstitial lung disease?

A

Idiopathic pulmonary fibrosis
Cryptogenic organising pneumonia
Non-specific interstitial pneumonia

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13
Q

Investigations of interstitial lung diseases.

A

CXR: abnormal - bilateral diffuse pulmonary infiltrates

Look at systemic features - these could indicate a systemic cause

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14
Q

Are interstitial lung diseases obstructive or restrictive?

A

Restrictive

They have a normal FEV1/FVC ratio

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15
Q

What is idiopathic pulmonary fibrosis?

A

An autoimmune condition where the lungs become fibrosed

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16
Q

What happens to patients with idiopathic pulmonary fibrosis?

A

Scarring limits the patient’s ability to respire

Usually they end up getting a terminal infection and become hypoxic

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17
Q

What is the link between rheumatoid arthritis and interstitial lung disease?

What is a syndrome some patients with RA and ILD get? Describe.

A

RA can cause inflammation in the lungs, which leads to fibrosis - interstitial lung disease

Caplan’s syndrome: a combination of RA and pneumoconiosis (from occupational exposure) which manifests as pulmonary nodules

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18
Q

What is meant by granulomatous disease?

A

A disease involving the formation of granulomas, which impair the function of the organ affected

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19
Q

What is a granuloma?

A

A granuloma is a collection of WBC’s (mononuclear cells and macrophages), surrounded by lymphocytes, plasma cells, mast cells, fibroblasts, and collagen.

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20
Q

What is sarcoidosis?

A

A multi-system disease that causes granulomas to develop in various places in the body

The immune system goes into “overdrive”, where the body starts to attack its own tissues and organs.

The resulting inflammation then causes granulomas to develop in the organs.

Unknown cause

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21
Q

Which organs are most commonly affected in sarcoidosis?

A

Lung
Skin
Eyes

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22
Q

Are the granulomas in sarcoidosis caseating or non-caseating?

A

Non-caseating

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23
Q

What are the clinical features of sarcoidosis?

A

Incidental finding

Erythema nodosum
Swinging fever
Lupus pernio
Malaise, weight loss

Pulmonary involvement:

  • Cough
  • SOB
  • Wheeze
  • Fine crackles
  • Progressive fibrosis
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24
Q

Investigations for sarcoidosis?

A

Chest + bone x-rays

Bloods

ECG

Lung function test

Tissue biopsy

Bronchoalveolar lavage

USS

CT/MRI

25
Q

What is lymphopenia?

A

Low lymphocyte numbers

26
Q

What blood tests do you do for sarcoidosis? What results would you expect?

A
  • raised ESR
  • lymphopenia
  • raised ACE
  • raised calcium
  • raised immunoglobulins
27
Q

What would you see on:

  • chest x-rays
  • bone x-rays

in sarcoidosis?

A

Chest
At later stages may see pulmonary infiltrates and signs of fibrosis, honeycomb lung

Bone
Punched out lesions in terminal phalanges

28
Q

What would an ECG show in sarcoidosis?

A

Arrhythmias

Bundle branch block

29
Q

What would you look for in a tissue biopsy of someone with sarcoidosis?

A

Non-caseating granulomas

30
Q

What would a bronchoalveolar lavage show in sarcoidosis?

A

Raised lymphocyte levels: in active disease only

31
Q

What areas would you ultrasound in sarcoidosis and what would you see?

A

Liver, spleen and kidneys

You may see nephrocalcinosis
Hepatosplenomegaly

32
Q

What 3 things do you need to have before you can diagnose sarcoidosis?

A

Clinical features
Radiology
Tissue sample

33
Q

Management of patients with acute sarcoidosis?

A

Bed rest

NSAIDs

34
Q

Sarcoidosis most of the time goes away within three years.

True or false?

A

True!

In a small minority of patients it persists
Only 2-5% of cases will have a reduced life-expectancy

35
Q

All patients with sarcoidosis require treatment?

True or false

A

False

Patients in their early stages won’t need treatment

36
Q

In sarcoidosis, when should you prescribe steroids?

And which type of steroids?

A

Corticosteroids

  1. If they have parenchymal lung disease
  2. Uveitis
  3. Hypercalcaemia
  4. Neurological or cardiac involvement
37
Q

Name a corticosteroid used to treat sarcoidosis?

A

Prednisolone

38
Q

What drugs can you use to treat sarcoidosis?

A

Methylprednisolone
DMARDs (methotrexate)
Ciclosporin

39
Q

What is bihilar lymphadenopathy?

A

Enlargement of the lymph nodes of the pulmonary hila

40
Q

What causes bihilar lymphadenopathy?

A 
Sarcoidosis
Infection
Malignancy
Organic dust disease, eg. Silicosis
Extrinsic allergic alveolitis
41
Q

Name some granulomatous interstitial lung diseases?

A

Sarcoidosis

Granulomatosis with polyangiitis (wegners)

42
Q

What is Granulomatosis with polyangiitis?

A

A type of vasculitis that causes lesions in the upper respiratory tract, the lungs and the kidneys

Inflammation of the small blood vessels

43
Q

Who gets Granulomatosis with polyangiitis?

A

Middle aged and elderly mostly

44
Q

What causes Granulomatosis with polyangiitiss?

A

Unknown

Could be linked with silica or Staph aureus infection

45
Q

What antibodies are found in most patients with Granulomatosis with polyangiitis?

A

cANCA

46
Q

What is an ANCA?

A

Anti-neutrophil cytoplasmic antibody

47
Q

Symptoms of Granulomatosis with polyangiitis?

A

Presents very differently in different people

Tiredness
Loss of appetite
Aching muscles + joints

Organ specific symptoms

48
Q

What are the features of Granulomatosis with polyangiitis affecting the lung?

A

Breathlessness, wheeze, dry cough, coughing up blood

49
Q

What organs can be affected in Granulomatosis with polyangiitis?

A 
Lungs
Skin
Eyes
Nerves
Bowels
50
Q

How do you diagnose Granulomatosis with polyangiitiss?

A

Blood tests: show signs of inflammation, and usually cANCA positive

Biopsy

51
Q

Treatment of Granulomatosis with polyangiitis?

A

Prednisolone: steroids to get disease under control

DMARDs: methotrexate

Plasmapheresis in severe cases

Biologics

52
Q

What is Goodpasture’s syndrome?

A

Autoimmune condition where antibodies attack the basement membranes in lungs + kidneys

53
Q

Which antibodies are involved in Goodpasture’s syndrome?

A

Anti-GBM (glomerular basement membrane)

54
Q

What are the clinical features of Goodpasture’s syndrome?

A

Acute glomerulonephritis
- protein + haematuria

Diffuse pulmonary haemorrhage

  • coughing up blood
  • SOB
  • uraemia
  • high BP
55
Q

Investigations of Goodpasture’s syndrome?

A

CXR: pulmonary infiltrates in lower zones

Kidney biopsy: signs of glomerulonephritis

56
Q

Is Goodpasture’s syndrome life threatening?

A

Yes, can quickly result in permanent lung and kidney damage

57
Q

Treatment of Goodpasture’s syndrome?

A

Immunosuppressants
Corticosteroids
Plasmapheresis

58
Q

What is plasmapheresis?

A

Removal of harmful antibodies from the blood

Like dialysis sort of

Respiratory (17 decks)

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