SARCOIDOSIS

Question 1

Define sarcoidosis.

Answer 1

Abnormal collection of inflammatory cells which form a granuloma. Can affect any organ but lungs and lymph nodes are the most commonly affected.

Question 2

What are the causes of sarcoidosis?

Answer 2

The exact cause of sarcoidosis is not know.

Genetics -
some heritability however, differs across races. Black more likely to have family member with sarcoidosis.

Infectious agents -
Mycobacteria, fungi, borrelia and rickettsia

Autoimmune - some evidence for Th1 lymphokine prevalence.

Question 3

What are the cell types that accumulate to form a granuloma?

Answer 3

Monocytes
Macrophages
Activated T-lymphocytes.

Question 4

The production of which inflammatory mediators is increased during the granulomatous inflammation? (Name at least 3)

Answer 4

TNF 
IFN-γ 
IL-2
IL-8
IL-10
IL-12
IL-18
IL-23 
TGF-β

Question 5

Is the immune response mediated by Th1 or Th2?

Answer 5

Th1

Question 6

How do patient with sarcoidosis respond to antigen challenges such as tuberculin?

Answer 6

The immune response is suppressed. This is paradoxical.

Question 7

What is the paradoxical suppression of immune response to antigen challenges coupled with a hyper-reactive immune response causing the granulomas suggestive of?

Answer 7

A state of anergy

Question 8

What is anergy?

Answer 8

An inability of the immune system to mount an appropriate response.

Question 9

What are patients with sarcoidosis more at risk of developing?

Answer 9

Infection

Cancer

Question 10

How might a patient with suspected sarcoidosis present? (Name at least 3 symptoms)

Answer 10

Fatigue
Weight loss
Shortness of breath
Dry cough
Rarely hemoptysis
Joint aches and pains
Dry eyes
Blurry vision
Skin lesions

Question 11

What signs might a patient with suspected sarcoidosis exhibit on examination?

Answer 11

Rashes
Skin nodules
Erythema nodosum (red inflammation of fat cells under the skin)
Raised JVP (from RVF as a result of pulmonary arterial hypertension)
Peripheral oedema
Reduced breath sounds
Abnormal breath sounds

Question 12

What is Lofgren syndrome?

Answer 12

Type of acute sarcoidosis. More frequent in scandinavian, irish, african and puerto rican women.
Characterised by erythema nodosum, bilateral hilar lymphadenopathy and joint pain.