Sarcoidosis Flashcards
It is a multi-system granulomatous disorder.
What are granulomas?
What 2 places does it affect the most?
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
Epidemiology:
What age group does it affect more?
What sex is it more common in?
What ethnicity does it affect more?
20-40
Women>Men
Afro-Caribbean people
How are most cases discovered?
Incidentally after routine CXR
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
SOB
Persistent dry cough
Wheeze
Chest pain
Clinical Features - Lymph:
- What is BHL?
- Where else may lymph nodes be found?
Bilateral hilar lymphadenopathy
Cervical and/or submandibular
Clinical Features - Skin:
- What is found on the legs?
- What lupus pernio? Where is it found?
Erythema nodosum - look up
Hard purple lesions on the face
Clinical Features - Eyes:
What diseases happen in the eyes? - 3
Uveitis
Conjunctivitis
Optic neuritis
Clinical Features - Liver:
- How does it affect the liver? - 3
Liver nodules
Cirrhosis
Cholestasis
Clinical Features - Heart:
How does it cause heart block and dilated cardiomyopathy?
Formation of granulomas in the myocardium.
Clinical Features - Kidneys:
Why do kidney stones form?
It causes hypercalcaemia
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?
Encephalopathy
Diabetes insipidis
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Mononeuritis multiplex
Bells palsy - it can cause other CN palsies
Clinical features - Bones and muscles:
What 3 manifestations happen?
Arthralgia - painful bones
Arthritis - painful joints
Myopathy
Lofgren syndrome:
This happens in acute sarcoidosis and FEVER is also present.
What is the triad in this? - P, E, B
Polyarthralgia
Erythema nodosum
BHL - bilateral hilar lymphadenopathy
Pulmonary sarcoidosis:
What are the 3 manifestations of sarcoidosis in the lungs?
Pulmonary nodules
Pulmonary fibrosis - ILD before fibrosis
Mediastinal lymphadenopathy
DDx for bilateral lymphadenopathy:
Another granulomatous disease that can cause BHL?
What types of cancer may lead to this?
TB
Lymphoma
Non-small cell lung cancer
Breast cancer
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?
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
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?
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
Investigations:
What are lung function tests used for?
What type of pattern are you likely to see?
To monitor disease progression
Restrictive but can be mixed
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?
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
Investigations:
Why is a CT/MRI done?
Looking for neurosarcoidosis or assessing severity of the pulmonary disease.
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?
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
