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Respiratory > Diffuse parenchymal lung disorders / interstitial lung diseases > Flashcards

Diffuse parenchymal lung disorders / interstitial lung diseases Flashcards

1
Q

What are interstitial lung diseases?

A

Group of diseases with bilateral diffuse lung injury and inflammation that can progress to lung fibrosis
Involves interstitial space, alveoli, bronchioles and blood vessels

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2
Q

What is the common presentation?

A

Dyspnoea
Persistant non productive cough
Abnormal CXR- bilateral diffuse pulmonary infiltrates
Pulmonary symptoms

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3
Q

What is sarcoidosis?

A

Multi-system granulomatous disorder affecting any organ system, but predominantly the lymph nodes and lung

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4
Q

What genes mutations are associated with sarcoidosis?

A

HLA-DRB1 and DQB1 alleles

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5
Q

What type of granuloma is present in sarcoidosis?

A

non-caseating sarcoid granuloma: focal accumulation of epithelial cells, macrophages and lymphocytes.

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6
Q

Describe the pathophysiology of sarcoidosis?

A

T cells are removed in the lung and trapped in granulomas
–>
Depressed cell mediated reactivity to antigens such as tuberculin and candida albicans + overall lymphopenia with low circulating T cells

Increased number of cells (esp. CD4 helper) in bronchoalveolar lavage

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7
Q

Describe epidemiology of sarcoidosis

A

age = 20-40
F>M
Afro Caribbean > Caucasian

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8
Q

How does sarcoidosis usually present

A

Often incidental finding on CXR

Acute sarcoidosis presents with erythema nodosum, and polyarthralgia

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9
Q

What is erythema nodosum?

A

red lumps caused by inflammation of fat cells

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10
Q

What is polyarthralgia?

A

Multiple achy joints

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11
Q

What pulmonary features are present in sarcoidosis?

A

CXR – bilateral hilar lymphadenopathy ± pulmonary infiltrates or fibrosis

Progressive dyspnoea
Dry cough
decreased exercise tolerance
Chest pain

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12
Q

What non pulmonary signs are there?

A

Literally everything man

But most commonly: Lymphadenopathy, Hepatomegaly, Splenomegaly, Uveitis

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13
Q

What is the differential diagnosis of Bi-hilar lymphadenopathy?

A

Sarcoidosis
Infection e.g. TB, mycoplasma
Malignancy e.g. lymphoma, carcinoma, mediastinal tumours
Organic dust disease e.g. silicosis, berylliosis
Extrinsic allergic alveolitis (hypersensitivity pneumonitis)
Histocytosis X

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14
Q

What different types of granulomatous disease are there?

A 
Infections 
Autoimmune
Vasculitis 
Organic dust disease
Idiopathic 
Hypersensitivity pneumonitis
Histiocytosis X
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15
Q

What blood test results are typical for a patient with sarcoidosis?

A 
Raised ESR
Lymphopenia 
Raised serum ACE in 60% (non specific) 
Raised LFTs, 
Raised calcium 
Raised IGs
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16
Q

What will urine analysis show for a patient with sarcoidosis?

A

Raised calcium

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17
Q

Chest Xray is abnormal in 90% what are stages 0-4 for sarcoidosis

A 
0- normal 
1- Bi-hilar lymphadenopathy 
2- BHL + pulmonary infiltrate 
3- pulmonary infiltrate alone 
4- Pulmonary fibrosis with honey combing and pleural involvement
18
Q

What do lung function tests show for patients with sarcoidosis?

A

generally show restriction and reduced volume

19
Q

What spirometry results would indicate restrictive lung disease

A

FVC <0.8 of expected value

FEV1 >0.8 of FVC

20
Q

A positive tissue biopsy in sarcoidosis is diagnostic. What would a tissue biopsy show?

A

shows non-caseating granuloma – lung, liver, lymph nodes, skin nodules

21
Q

What does broncheoalveolar lavage show in patients with sarcoidosis?

A

Bronchoalveolar lavage (BAL) – shows increased lymphocytes in active disease, increased neutrophils in pulmonary fibrosis

22
Q

How would you treat BHL associated with sarcoidosis?

A

BHL alone doesn’t need treatment

23
Q

For acute sarcoidosis bed rest and NSAIDs are likely to induce a spontaneous recovery.
What are indications that a patient with sarcoidosis needs to be put on oral prednisolone? (corticosteroid)

A

Parenchymal lung disease
Uveitis
Hypercalcaemia
Neurological/cardiac involvement

24
Q

If sarcoidosis is not controlled by prednisolone what progressive treatment options are there?

A

Iv methylprednisolone or immunosuppressants (methotrexate, hydroxychloroquine, ciclosporin, cyclophosphamide)

25
Q

What are common dose related side effects of methotrexate?

A

Mucosal damage and bone marrow suppression

26
Q

What contraindications are there for methotrexate?

A

Teratogenic so avoid in pregnancy

27
Q

How does methotrexate act?

A

Inhibits conversion of folate into active form–> inhibits DNA production and hence cell division

Should prescribe folic acid with it!!

28
Q

Idiopathic pulmonary fibrosis, what is it?

A

A type of idiopathic interstitial pneumonia.

Inflammatory cell infiltrate and pulmonary fibrosis of unknown cause.

29
Q

Idiopathic pulmonary fibrosis symptoms?

A 
Dry cough 
Exertional dyspnoea 
Malaise 
Weight loss 
Arthralgia
30
Q

Idiopathic pulmonary fibrosis signs?

A

Cyanosis
Finger clubbing
Fine end inspiratory crackles

31
Q

Idiopathic pulmonary fibrosis complications?

A

Respiratory failure

Increased risk of cancer

32
Q

Blood test results for patient with idiopathic pulmonary fibrosis

A

ABG: PaO2 low, if severe then PaCo2 is also high
CRP raised
Ig’s raised
ANA and rheumatoid factors (very unspecific but indicative of disease)

33
Q

CXR for patients with idiopathic pulmonary fibrosis

A

Lung volume decreased
Bi-lateral lower zone reticulo-nodular shadows
Honey comb lung

CT shows the same but more sensitive

34
Q

What would spirometry show for idiopathic pulmonary fibrosis?

A

Restrictive changes
FVC< 0.8 of expected value
FEV1>0.8 FVC

35
Q

Lung biopsy may be required for a diagnosis what are the histological changes observed called?

A

Usual interstitial pneumonia

36
Q

Treatment for idiopathic pulmonary fibrosis?

A 
O2 
Supportive treatment 
Palliative care 
Opiates 
pulmonary rehab 
Lung transplant is feasible
37
Q

What is interstitial lung disease?

A

Characterized by chronic inflammation and/or progressive interstitial fibrosis

38
Q

Describe the pathology of interstitial lung disease

A

Fibrosis and remodelling of the interstitium
Chronic inflammation
Hyperplasia of type 2 pneumocytes

39
Q

Clinical presentation of interstitial lung disease?

A 
Dyspnoea on exertion 
Non productive paroxysmal (sudden intense recurrence) cough
Abnormal breathing sounds
Abnormal Xray and CT
Spirometry shows restriction, 
reduced tCO

(everything you would expect tbh)

40
Q

Name 4 occupational interstitial lung diseases

A

Asbestosis, berylliosis, silicosis, cotton workers lung

41
Q

What drugs can cause occupational interstitial lung disease?

A 
Nitrofurantoin (abx for bladder infection)
Bleomycin 
Amiodarone (anti- arrhythmic drug) 
Sulfasalazine (RA drug) 
Busulfan (chemo)
42
Q

What are all the different causes of interstitial lung disease?

A

Occupational (Asbestosis)
Drugs (amiodarone)
Hypersensitivity (Hypersensitivity pneumonitis)
Infections (e.g. viral)
GORD
Those associated with systemic disorders (e.g. sarcoidosis, SLE)
Idiopathic (idiopathic pulmonary fibrosis)

Respiratory (15 decks)

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