Restrictive: pulmonary fibrosis, sarcoidosis, pulmonary hypertension

Question 1

Define pulmonary fibrosis?

Answer 1

Replacement of elastic and functional lung tissue with non-functional scar tissue.

Question 2

Causes of pulmonary fibrosis?

Answer 2

Lung damage due to:
- pneumonia
- tuberculosis
- infarction

Connective tissue disease:
- SLE
- RA
- Sjögren’s syndrome
- Systemic sclerosis

Upper (CHEATS):
Cystic fibrosis
Hypersensitivity pneumonitis
Extrinsic allergic alveolitis
Ankylosing spondylitis
Tuberculosis
Sillicosis/sarcoidosis

Lower (RAIDS):
Rheumatoid arthritis
Asbestosis
Idiopathic
Drugs
Scleroderma

Question 3

Presentation of pulmonary fibrosis?

Answer 3

SOB on exertion
Dry cough
fatigue

Cyanosis
Clubbing
Cachexia
Fine end-inspiratory crackles (unilateral, basal, apical, or global)
Signs of connective tissue disease.

Question 4

Diagnosis/IVx of pulmonary fibrosis?

Answer 4

CXR (reticular and nodular shadowing, bilateral hilar lymphadenopathy -sarcoidosis)

HRCT -GOLD STANDARD
(honeycomb, ground-glass change, traction bronchiectasis)

Bloods:
- FBC
- U&Es, LFTs, bone profile
- CRP
- rheumatoid factor
- autoimmune screen (ANA, ANCA, ENA)
- Scl-70 antibodies (systemic sclerosis)
- Serum angiotensin-converting enzyme – Sarcoidosis

Question 5

Management of pulmonary fibrosis?

Answer 5

Lifestyle
Pulmonary rehab
Monitoring
Oxygen therapy
Antifibrotic drugs
Lung transplant -definitive cure for severe disease

Question 6

Define/causes of sarcoidosis?

Answer 6

Chronic granulomatous disorder that affects multiple organs with widespread inflammatory changes.

Unknown cause. May be related to an abnormal immune response.

Question 7

Presentation of sarcoidosis?

Answer 7

Fatigue
Arthralgia
Lymphadenopathy
Dry Cough
Dyspnoea
Uveitis

Can have a range of symptoms.

Neurological: Meningitis, peripheral neuropathy, bilateral Bell’s palsy

Ocular: Uveitis, keratoconjunctivitis sicca

Cardiac: Arrhythmias, restrictive cardiomyopathy

Abdominal: Hepatomegaly, splenomegaly, renal stones

Dermatological: Erythema nodosum, lupus pernio

Question 8

Diagnosis/IVx of sarcoidosis?

Answer 8

CXR or HRCT
Stage 1 - Bilateral hilar lymphadenopathy (BHL)
Stage 2 - BHL with peripheral infiltrates
Stage 3 - Peripheral infiltrates alone
Stage 4 - Pulmonary fibrosis

12 lead ECG

Bloods
- raised ESR, ACE, calcium levels, urea and creatinine
- reduced lymphocytes

Question 9

Management of sarcoidosis?

Answer 9

Acute sarcoidosis:
- bed rest
- NSAIDs

Corticosteroid tx (1ST LINE):
- oral or topical depending on severity

Immunosuppressants (2ND LINE)

Biologics (3RD LINE)

Bilateral hilar lymphadenopathy alone:
- self-limiting
- doesn’t require tx

Lung transplant

Question 10

Define/cause of pulmonary hypertension?

Answer 10

Increase in mean pulmonary arterial pressure (>25mmHg)

Cause:
- left heart disease
- hypoventilation
- left heart disease
- lung disease
- blood clots
- conditions that cause the smaller pulmonary vessels to narrow

Question 11

Presentation of pulmonary hypertension?

Answer 11

SOB
tachycardia
syncope
angina
oedema
raised JVP
presence of an S3 sound
loud P2
pansystolic murmur (tricsupid regurg)
end-diastolic murmur (pulmonary regurg)

Question 12

Diagnosis/IVx of pulmonary hypertension?

Answer 12

ECG (P pulmonale -indicates right ventricular hypertrophy)

Echocardiogram (increase right heart pressure)

Right heart catheterisation -GOLD STANDARD
- mean pulmonary artery pressure >25mmHg

Question 13

Management of pulmonary hypertension?

Answer 13

Treat underlying condition.

Reduce pulmonary vascular resistance
- long term oxygen therapy
- Nifedipine
- Sildenafil
- prostacycline analogues
- bosentan

Manage heart failure

Anticoagulants
Diuretics

Heart/lung transplant