Sarcoidosis

Question 1

What is sarcoidosis? What are some extra-pulmonary manifestions of it?

Answer 1

• a chronic granulomatous disorder (granulomas are inflammatory nodules full of macrophages). The cause of these granulomas is unknown.
• erythema nodosum and lymphadenopathy

Question 2

In who is sarcoidosis more common in?

Answer 2

• In women
• Afro-Caribbean ethnicity
• aged 20-39 or around 60

Question 3

What is erythema nodosum?

Answer 3

• nodules of inflamed subcutaneous fat on the shins
• presents as raised, red, tender, painful nodules across both shins.

Question 4

What is lupus pernio?

Answer 4

• specific to sarcoidosis
• presents with raised purple skin lesions, often on theh cheeks and nose

Question 5

How does sarcoidosis affect the different organs in the body? (Lungs, systemic symptoms, liver, eyes, heart, kidneys, CNS, PNS, Bones)

Answer 5

Lungs:
- mediastinal lymphadenopathy
- pulmonary fibrosis
- pulmonary nodules

Systemic symptoms:
- fever
- fatigue
- weight loss

Liver:
- liver nodules
- cirrhosis
- cholestasis

Eyes:
- Uveitis
- Conjunctivitis
- optic neuritis

Heart:
- Bundle branch block
- heart block
- myocardial muscle involvement

Kidneys:
- kidney stones
- nephrocalcinosis
- interstitial nephritis

CNS:
- nodules
- pituitary involvement (Diabetes)
- Encephalopathy

PNS:
- Facial nerve palsy
- mononeuritis multiplex

Bones:
- arthalgia
- arthritis
- myopathy

Question 6

What is Lofgren’s syndrome?

Answer 6

Lofgren’s syndrome refers to a specific presentation of sarcoidosis with a classic triad of symptoms:

1) Erythema nodosum
2) Bilateral hilar lymphadenopathy
3) Polyarthralgia (joint pain in multiple joints)

Question 7

What are the top differentials for varied presenting features of sarcoidodis? (6)

Answer 7

• TB
• Lymphoma
• hypersensitivity pneumonitis
• HIV
• Toxoplasmosis
• Histoplasmosis

Question 8

What investigations would you do to confirm the diagnosis?

Answer 8

• FBC- raised ACE, and calcium
• CXR- hilar lymphadenopathy
• HRCT- hilar lyphadenopathy and pulmonary nodules
• MRI- shows CNS involvement
• PET scan- active inflammation in affected areas

Question 9

What are the histological findings of sarcoidosis?

Answer 9

• non-caseating granulomas with epithelioid cells

Question 10

What are some other tests you would do to determine which organs are affected?

Answer 10

• U&Es
• LFTs
• Opthalmology
• ECG and echo
• US- for liver and kidney involement

Question 11

What is the management for sarcoidosis?

Answer 11

• conservative management for patients with no or mild symptoms
• oral steroids- first-line treatment
• bisphosphonates to protect against osteoporosis whilst on long term steroids
• methotrexate as the second-line treatment
• lung transplant

Question 12

What is the prognosis of sarcoidosis?

Answer 12

Sarcoidosis spontaneously resolves in around half of patients, usually within two years. In some patients, it progresses to pulmonary fibrosis and pulmonary hypertension. Overall mortality is less than 10%.