Sarcoidosis

Question 1

It is a multi-system granulomatous disorder.

What are granulomas?

What 2 places does it affect the most?

Answer 1

A granuloma is a structure formed during inflammation that is found in many diseases. It is a collection of immune cells known as macrophages. Granulomas form when the immune system attempts to wall off substances it perceives as foreign but is unable to eliminate.

Lungs
Lymph nodes

Question 2

Epidemiology:

What age group does it affect more?

What sex is it more common in?

What ethnicity does it affect more?

Answer 2

20-40

Women>Men

Afro-Caribbean people

Question 3

How are most cases discovered?

Answer 3

Incidentally after routine CXR

Question 4

Clinical Features - 90% of patients will have pulmonary disease.

This is why it is managed by resp doctors.

What sort of symptoms will they have? - 4

Answer 4

SOB
Persistent dry cough
Wheeze
Chest pain

Question 5

Clinical Features - Lymph:

• What is BHL?
• Where else may lymph nodes be found?

Answer 5

Bilateral hilar lymphadenopathy

Cervical and/or submandibular

Question 6

Clinical Features - Skin:

• What is found on the legs?
• What lupus pernio? Where is it found?

Answer 6

Erythema nodosum - look up

Hard purple lesions on the face

Question 7

Clinical Features - Eyes:

What diseases happen in the eyes? - 3

Answer 7

Uveitis
Conjunctivitis
Optic neuritis

Question 8

Clinical Features - Liver:

• How does it affect the liver? - 3

Answer 8

Liver nodules
Cirrhosis
Cholestasis

Question 9

Clinical Features - Heart:

How does it cause heart block and dilated cardiomyopathy?

Answer 9

Formation of granulomas in the myocardium.

Question 10

Clinical Features - Kidneys:

Why do kidney stones form?

Answer 10

It causes hypercalcaemia

Question 11

Clinical Features - Neurosarcoidisis (<10%):

The patient ends up having nodules in the brain.

• What could this cause generally?
• What may happen if it happen in the pituitary gland?

Clinical Features - PNS:

• One nerve can be affected by a nodule. What is this called?
• What type of palsy may happen in the face?

Answer 11

Encephalopathy

Diabetes insipidis

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Mononeuritis multiplex

Bells palsy - it can cause other CN palsies

Question 12

Clinical features - Bones and muscles:

What 3 manifestations happen?

Answer 12

Arthralgia - painful bones
Arthritis - painful joints
Myopathy

Question 13

Lofgren syndrome:

This happens in acute sarcoidosis and FEVER is also present.

What is the triad in this? - P, E, B

Answer 13

Polyarthralgia
Erythema nodosum
BHL - bilateral hilar lymphadenopathy

Question 14

Pulmonary sarcoidosis:

What are the 3 manifestations of sarcoidosis in the lungs?

Answer 14

Pulmonary nodules
Pulmonary fibrosis - ILD before fibrosis
Mediastinal lymphadenopathy

Question 15

DDx for bilateral lymphadenopathy:

Another granulomatous disease that can cause BHL?

What types of cancer may lead to this?

Answer 15

TB

Lymphoma
Non-small cell lung cancer
Breast cancer

Question 16

Investigations:

Bedside:
- Why do an ECG?

Bloods:

• What is used to test for inflammation?
• What type of WBC is usually low?
• Why is there hypercalcaemia?
• Why do you look at LFT’s and U+E’s?
• Why is serum ACE measured?

Answer 16

1,25-dihydroxyvitamin D3 (Calcitriol) leads to increased absorption of calcium in the intestine and to increased resorption of calcium in the bone.

May show arrhythmia or bundle branch blocks due to nodules

ESR/CRP

Lymphocytes - lymphopenia

Due to the uncontrolled synthesis of 1,25-dihydroxyvitamin D3 (Calcitriol) by macrophages.

There may be liver and kidney involvement
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ACE is produced by epithelioid cells surrounding granulomas.- its made in the lungs remember

Question 17

Investigations:

• Why is a 24 hr urine done?

CXR - There are 4 stages:
Stage 0 is normal on CXR.

• What 1 feature is found in stage 1? Do they need Rx?
• What extra feature is found in stage 2?
• What new disease ends up manifesting in stage 3? What feature is lost at this stage?
• What happens in stage 4 meaning the patient begins to really struggle?

Answer 17

1 - BHL - No Rx is needed as they self-resolve

2 - BHL + Pulmonary infiltrates

3 - ILD - interstitial lung disease - NO BHL

4 - Pulmonary fibrosis, bulla formation, pleural involvement

Question 18

Investigations:

What are lung function tests used for?

What type of pattern are you likely to see?

Answer 18

To monitor disease progression

Restrictive but can be mixed

Question 19

Investigations:

A biopsy is the only thing that can confirm the diagnosis:

How is a biopsy taken? - 4

Why may a USS be done?

Bone XR:

• What bones are typically affected?
• What does it look like on XR?

Answer 19

Flexible bronchoscopy with bronchoalveolar lavage
Fine needle aspiration
Lymph node biopsy via EBUS or mediastinoscopy
Skin biopsy

Looking for nephrocalcinosis or hepatosplenomegaly

Phalanges
Punched out appearance

Question 20

Investigations:

Why is a CT/MRI done?

Answer 20

Looking for neurosarcoidosis or assessing severity of the pulmonary disease.

Question 21

Management:

Stage 0-1 doesn’t need Rx.

Indications for corticosteroid use? - 4
What steroid is given?

What is given in severe disease?

What is the final option?

Why does osteoporosis at risk of developing?
What is given for osteoporosis protection?

Answer 21

Lung transplant

Symptomatic lung disease
Uveitis - the risk of sight loss
Hypercalcaemia
Neurological or cardiac involvement

Prednisolone

IV steroids (methylprednisolone)
Immunosuppressants (methotrexate)

Increase bone resorption due to release of Calcitriol
Biphosphonates