Pulmonary Fibrosis + Bronchiectasis

Question 1

Types of PF

Answer 1

Replacement fibrosis = secondary to lung damage

Focal fibrosis = response to irritants

Idiopathic PF = can cause diffuse parenchymal lung disease

Question 2

Pathology of IPF

Answer 2

Excess collagen in lung

Type 1 alveoli = squamous cells, form near continuous barrier between air + connective tissue

Type 2 alveoli = have microvilli, secrete surfactant, can divide + become type 1 or type 2

Between type 1 + type 2 alveoli is interstitial tissue containing macrophages + fibroblasts

When alveoli is damaged, type 1 cells release TGFB1, stimulated fibroblasts to differentiate into myofibroblasts -> these produce reticular + elastic fibres

Type 2 cells over-proliferate in fibrosis, myofibroblasts don’t undergo apoptosis

Thickened interstitial layer -> causes problems with ventilation + oxygenation (restricted lung expansion)

Decreased total lung capacity

Decreased forced vital capacity

Decreased FEV1

Loss of alveoli causes fluid filled cysts (honeycombing)

Question 3

RF for PF

Answer 3

Smoking

Bird + animal droppings

Herpes virus

GORD

Fam hx

Radiation

Drugs - chemo, amiodarone, methotrexate, nitrofurantoin

Question 4

What other conditions is PF associated with?

Answer 4

Thyroid disease

Systemic sclerosis

RA

SLE

Autoimmune liver disease

Question 5

Investigation results for PF

Answer 5

Low O2, CO2 retention if severe I

ncreased CRP

CXR: decreased lung volume, bilateral lower zone reticulonodular shadows, honeycomb lung

CT is essential for diagnosis

Spirometry = decreased FVC, FEV1/FVC ration equal or increased

Question 6

Complications of PF

Answer 6

Respiratory failure, increased risk of lung cancer

Question 7

Management of PF

Answer 7

Pirfenidone or nintedanib

Supportive therapy

Lung transplant

Question 8

Causes of bronchiectasis

Answer 8

Post infection

No known cause

Question 9

RF for bronchiectasis

Answer 9

CF, Kartageners TB, pertussis, measles, pneumonia

Bronchial obstruction, aspergillosis, hypogammaglobulinaemia, gastric aspiration

Question 10

S+S of bronchiectasis

Answer 10

Winter exacerbation - fever, cough, sputum, pleuritic chest pain + SOB

Haemoptysis + wheeze

Clubbing, low pitched crackles + wheeze

Question 11

Investigations for bronchiectasis

Answer 11

CXR

Sputum MC+S

Spirometry (reversible obstruction)

CT for diagnosis

Question 12

Treatment of bronchiectasis

Answer 12

physio, abx, bronchodilators

Question 13

What are common causes of pneumonia + S+S of each

Answer 13

Staph = following flu

H influenza = if COPD L

egionella = dry cough, hyponatraemia, lymphopenia

Question 14

What is sarcoidosis?

Answer 14

Causes constitutional upset eg weight loss, fever, night sweats

Shows restrictive pattern on spirometry

Caused by non-caseating epitheloid granuloma deposits

Causes lung + skin problems

Question 15

Pathology of bronchiectasis

Answer 15

Permanent dilatation + thickening of airways associated with chronic cough, sputum production, bacterial colonisation + recurrent infection

Question 16

Complications of bronchiectasis

Answer 16

Progressive loss of lung function

RHF secondary to chronic resp disease

Urinary incontinence + sexual dysfunction

Chronic tiredness

Nutritional deficiency

Question 17

When can an acute infective exacerbation of bronchiectasis be diagnosed?

Answer 17

Acute deterioration with worsening cough +/- systemic upset

Presence of purulent sputum

Question 18

Conditions leading to lung transplantation

Answer 18

COPD

CF

Idiopathic interstitial pneumonias

IPAH, Eisenmenger’s syndrome

Sarcoidosis

Question 19

What are sicca symptoms?

Answer 19

Dry eyes + dry mouth

Associated with Sjogren’s syndrome

Question 20

What are the types of intersitital lung disease?

Answer 20

Asbestosis

Sarcoidosis

IPF

RA associated

Drug induced

Pneumoconiosis

Question 21

What changes do you get on CXR from interstitial lung disease?

Answer 21

Reticulodular changes = lines and dots from fibrosis

Volume loss

Specific distribution:

Upper lobe fibrosis (due to increased ventilation) = TB, hypersensitive pneumonitis, sarcoidosis

Lower lobe (perfuse more than you ventilate) = IPF, drugs

Question 22

What CT changes + signs will be present in idiopathic pulmonary fibrosis?

Answer 22

IPF = UIP pattern on CT (honeycombing - basal, bilateral and subpleural, on the peripheries)

50% will have clubbing

Have fine late basal inspiration crackles (velcro crackles)

SOBOE + dry cough

Question 23

What does the colour of sputum indicate?

Answer 23

Sputum is coloured due to neutrophils

Question 24

What is procalcitonin?

Answer 24

Early marker of infection

Question 25

How to reduce risk of exacerbation in bronchiectasis?

Answer 25

Physio, neb saline, azithromycin, nebulised abx (colisitin, tobramycin)