Restrictive thoracic disease Flashcards

1
Q

What are the causes of restrictive thoracic disease outside of the lung?

A

Skeletal- scoliosis of the spine, rib fractures, ankylosing spondylitis
Muscle weakness- Myopathy, neuropathy, myelopathy
Abdominal obesity- compression of thoracic content
Pregnancy

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2
Q

What causes restrictive thoracic disease within the lung?

A

Diffuse Parenchymal Lung Disease DPLD

Also know as Interstitial lung disease- ILD

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3
Q

WHat are the 5 different types of DPLD?

A

1) Acute
2) Episodic
3) Chronic due to occupational or environmental exposure
4) CHhronic with evidence of systemic disease
5) Chronic with no evidence of systemic disease

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4
Q

What is the pathophysiology of DPLD?

A

Disease of the alveolar structures.

Impaired alveolar gas exchange involving the alveolar arteriolar barrier.

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5
Q

Is carbon dioxide exchange impaired in DPLD and why?

A

No because ventilation is normal and carbondioxide is very soluble in the membranes

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6
Q

What are the causes of DPLD? (6 things)

A
Fluid in the alveolar air spaces 
Consolidation in alveolar air spaces 
Inflammatory infiltration of alveolar walls 
Pneumoconiosis
Carcinomatosis 
Type 1/3 hypersensitivity response
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7
Q

What are causes of fluid in the air spaces?

A

Cardiac pulmonary oedema due to left ventricular failure leading to back pressure in the left atrium, pulmonary veins and capillaries
Non cardiac pulmonary oedema. Leaky capillaries due to dysfunctional tight junctions from trauma or sepsis

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8
Q

What are the causes of consolidation in alveolar air spaces?

A

Infective pneumonia
Infarction due to PE or vasculitis
BOOP- bronchiolitis obliterons organising pneumonia

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9
Q

What causes alveolitis?

A

Extrinsic allergic alveolitis due to extrinsic antigen (type 3 hypersensitivity- IgG mediated)
Sarcoidosis- multisystem swelling of lymph nodes, inflammation of nerves and heart and erythema nodosum
Drug induced- Methatrexate, aminodarone, bleomycin
Toxic fumes- chlorine
Pulmonary fibrosis
Autoimmune- SLE

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10
Q

What are the 2 types of pneumoconiosis?

A

Fibrogenic- asbestosis, silicosis

Non fibrogenic- Iron, Tin Barium

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11
Q

Which adenocarcinomas are most likely to metastasise in the lung and cause carcinomatosis?

A

Broncho, breast, prostate, colon and stomach

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12
Q

What is the difference between asthma and eosinophilic DPLD?

A

In asthma, eosinophils infiltrate the bronchial tree whereas in eosinophilic DPLD the eosinophils infiltrate the alveolar tissue

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13
Q

What are the clinical signs of DPLD?

A
Breathless on exertion
Cough but no wheeze
Finger clubbing 
Inspiratory lung crackles
Central cyanosis 
Pulmonary fibrosis
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14
Q

What would you expect to find with spirometry in DPLD?

A

Restrictive pattern
Decreases FEV1 and FVC
Normal FEV1:FVC ratio of >75%
Normal peak flow

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15
Q

What test would you do to assess gas diffusion?

A

DLCO

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16
Q

Why are inhaled drugs not effective in DPLD?

A

Because you do not get a high enough concentration in the distal lung- particles are too large

17
Q

What tests can you do for DPLD?

A

Antibodies: avian, fungal, autoimmune
Serum ACE and Ca++ in sarcoidosis
CXR- bilateral alveolar infiltrates
Echo cardiogram if heart failure is suspected
CT scan
Bronchiolar lavage for infection
Transbronchial or thorascopic lung biopsy. Rarely indicated

18
Q

How can DPLD be treated?

A

Remove trigger factor
Immunosuppresants for reversible alveolitis
Steroids- prednisolone (1st line)
Azathioprine- steroid sparing (2nd line)

19
Q

How can ideopathic pulmonary fibrosis be treated?

A

Anti fibrotic agents: Pirfenidone and Nintedanib
Treat symptoms
Lung transplant