Restrictive Thoracic Disease

Question 1

What are the skeletal problems that can cause thoracic restriction?

Answer 1

Vertebrae - thoracic kyphoscoliosis, ankolosing spondylitis

Ribs - traumatic rib fracture

Question 2

What are the muscle weakness problems that can cause thoracic restriction?

Answer 2

Intercostal or diaaphragmatic weakness

myopathy, neuropathy, myelopathy

Question 3

What are the abdominal problems that can cause thoracic restriction?

Answer 3

Obesity

Ascites

Question 4

What do thoracic problems that originate from outwith the thoracic region result in?

Answer 4

Chronic alveolar under ventilation with a low PaO2 and a secreased SaO2, normal or low PaCO2 and reduced lung volume

Question 5

What are thoracic restrictions that result due to causes within the lungs?

Answer 5

Acute diffuse parenchymal lung disease (DLPD) is a group of lung diseases that affects the interstitium. Concerns alveolar epithelium, pulmonary capillary endothelium, basement membrane, perivasclar and perilymphatic tissues
Episodic DPLD
Chronic DLPD due to occupational or environmental agents/drugs
Chronic DLPD with evidence of systemic disease
Chronic DPLD with no evidence of systemic disease

Question 6

What is DLPD?

Answer 6

Disease of alveolar structures such as the alveolar walls and lumen (lung parenchyma)

Question 7

What is the pathophysiology of DLPD?

Answer 7

Impaired alveolar gas exchange
Alveolar barrier to oxygen exchange
CO2 exchange unimpaired as alveolar ventilaiton is normal
Decreased PaO2 and SaO2 and normal PaCO2

Question 8

What are the causes of DLPD?

Answer 8

Fluid in the alveolar space
Cardiac pulmonary oedema in alveolar walls and lumen due to raised pulmonary venous pressure
Non-cardiac pulmonary oedema - normal pulmonary venous pressure with leaky pulmonary capillaries due to sepssi or trauma

Question 9

What causes infective pneumonia?

Answer 9

Viral (Influenze, measles, chickenpox) bacteria (Pneumococcus, TB), fungal (HIV/pneumocystis), parasites (toxacara, filaris)

Question 10

What causes infarction?

Answer 10

PE, vasculitis

Question 11

What is BOOP?

Answer 11

Bronchiolitis obliterans organizing pneumonia

Question 12

What are other causes of restrictive lung disease?

Answer 12

Rheumatoid disease
Drugs
Cryptogenic

Question 13

What are allergic causes of alveolitis?

Answer 13

Farmers lung

Avian (birds)

Question 14

What is sarcoidosis?

Answer 14

Multi-system disease
Lymphadenoathy
Erythema nodosum

Question 15

What drugs/toxic gas that can induce alveolitis?

Answer 15

Amiodarone
Gold
Blemocycin
Chlorine

Question 16

What are autoimmune diseases that can cause alveolitis?

Answer 16

SLE
Polyarteritis
Wegners
Churg-strauss

Question 17

What are dust-diseases that can cause alveolitis?

Answer 17

Fibrogenic - asbestos/silicosis

Non-fibrogenic - siderosis (iron), stenosis (tin), baritosis (barium)

Question 18

What are carcinomas that can cause alveolitis?

Answer 18

Lymphatic/blood spread

Adenoa - bronchus, breast, prostate, colon, stomach

Question 19

What is the clinical syndrome of DLPD?

Answer 19

Breathless of exertion 
Cough but no wheeze 
Finger Clubbing 
Inspiratory lung crackles 
Central cyanosis 
Pulmonary fibrosis occurs at end stage response to chronic inflammation

Question 20

How is DPLD diagnosed?

Answer 20

History - occupation, drugs, pets, arthiritis
Reduced lung volumes - decreased FEV1, FVC but normal ratio of above 75%
Reduced gas diffusion (DLCO)
Arterial oxygen desaturation at rest or on exercise
Antibodies
Bilateral diffuse alveolar infiltrates on CXR
Echocardiogram to exclude heart failure
High resultion CT to identify inflammatory ground glass vs fibrotic nodular component of alveolar infiltrates in IPF
Breonchoalveolar lavage or induced sputum
Transbronchial or thorascopic lung biopsy

Question 21

What is the treatment of restrictive lung disease?

Answer 21

Remove trigger factor
Treat reversible alveolitis i.e. ground glass on high resolution CT
Oxygen in hypoxemic
Treatment of secondary pulmonary hypertension
Lung transplant