Advanced respiratory diseases

Question 1

How is a pulmonary embolus diagnosed?

How is the risk of PE assessed?

Answer 1

V/Q mismatch
Block on CT scan
D-dimer (one of the breakdown products of fibrin cap)
Duplex ultrasound
CXR

Virchows triad and Wells score

Question 2

Where is a PE most likely to originate?

Answer 2

DVT in leg particularly femoral, popliteal or tibial vein

Then pelvis

Then arm

Question 3

What are the signs and symptoms of PE

Answer 3

Dyspnoea
Tachycardia
Dry cough
Hypoxaemia
Pleuritic pain
Pleural rub
Haemoptysis
Syncope

Question 4

State the risk factors included within virchows triad

Answer 4

1. Hypercoagulability (genetics, pregnancy, oral contraceptives, malignancy, inflammatory. bowel disease, infection)
2. Circulatory stasis (immobility, pregnancy)
3. Vascular damage (smoking, surgery, hypertension)

Question 5

What is the treatment for PE

Answer 5

Anti-coagulation (heparin, warfarin, DOAC, factor Xa inhibitors)

(Removable) IVC filter

Question 6

What is sarcoidosis?

Answer 6

Sarcoidosis is a disease involving abnormal collections of inflammatory cells that form lumps known as granulomas.

It is a lung centred, multi-organ disease

Question 7

Describe the heterogeneity of sarcoidosis

Answer 7

1. NS lesions
2. Heart involvement: blocks, arrhythmia, failure
3. Eyes and glands
4. Liver and spleen nodules
5. Skin nodules
6. Systemic reaction

Question 8

Outline the pathophysiology of sarcoidosis

Answer 8

1. Sterile inflammation: activation of CD4+ T cells and macrophages
2. Activation process: unidentified antigen is inhaled into lungs. T cell receptors bind to antigen, APC and release of cytokines (IFN-g, IL-2, IL-12)
3. Macrophages make TNF-a and organise into granulomas
4. Chronic inflammation often turns into fibrosis

Question 9

How is sarcoidosis diagnosed?

Answer 9

Biopsy: findings could be chronic or in remission

Imaging would show

1. Lymphadenopathy: hilar>mediastinal
2. Lung nodules
3. Fibrosis

Question 10

What are the symptoms of sarcoidosis?

Answer 10

Asymptomatic
Cough and dyspnoea on exertion
Systemic symptoms: fatigue, sweats, weight loss, fever

Question 11

How is sarcoidosis managed?

Answer 11

Corticosteroids and immunosuppressants
Screen for multi-organ disease
Establish baseline PFT data

Question 12

Describe the aetiology of Cystic fibrosis

Answer 12

Autosomal recessive disease
CTFR protein = 1480amino acids long
Deletion or alteration of this protein leads to pathology
Change in F508(loss of phenylalanine)= most common

Leads to mucoid obstruction of exocrine glands

Question 13

Describe cystic fibrosis as a multi-system disease

Answer 13

Causes lung disease

Pancreatic insufficiency 
Malnourishment
Meconium ileus
Male infertility 
Osteoporosis

Question 14

How is cystic fibrosis screened and diagnosed?

What are its clinical findings?

Answer 14

Genetic screening
Sweat test

Wheezing
Haemoptysis
Rhonchi (snoring sounds from lung)
Bronchiectasis
Densities from mucous plugging
Shrunken , fibrotic lungs

Question 15

How can cystic fibrosis become complicated

Answer 15

ACUTE CAUSES
pseudomonas aeringosa and MRSA
flora changes with age (from gram positive to negative)

CHRONIC CAUSES
pseudomonas and B-cepacia
allergic bronchopulmonary aspergillosis

Haemoptysis, resp failure

Question 16

How is cystic fibrosis treated?

Answer 16

Bronchodilators
Airway clearance techniques
Mucus thinners

Others: corticosteroids, antibiotics, CFTR potentiators, lung transplant, pancreatic enzyme replacement