Granulomatous lung diseases - SARCOIDOSIS

Question 1

What is a granuloma?

& how is a granuloma formed?

Answer 1

= a mass or nodule composed of chronically inflamed tissue

A granuloma is formed by;
= the response of mononuclear phagocyte system

Question 2

why do granulomas form?

Answer 2

they form to confine a pathogen and limit the extent of surrounding inflammation and tissue destruction

Question 3

what is sarcoidosis?

Answer 3

a chronic disease caused by the ENLARGEMENT OF LYMPH NODES in many parts of the body and causes the appearance of NON-CASEATING GRANULOMATOUS.

Question 4

where in the body does sarcoidosis have an affect?

Answer 4

• most commonly just the lungs
• but it is has multi-system involvement;
  e. g. lymph nodes, joints, liver, skin, eyes, kidney, brain, nerve, heart

Question 5

how important is smoking when looking at sarcoidosis?

Answer 5

sarcoidosis is less common in smokers.

Question 6

what causes sarcoidosis?

Answer 6

• The cause is unknown.

However, there are a number of possibilities;
- e.g. imbalance of immune system with Type 4 hypersensitivity

Question 7

What are the 2 types of sarcoidosis?

Answer 7

1) acute sarcoidosis

2) chronic sarcoidosis

Question 8

what are the symptoms of sarcoidosis?

Answer 8

• enlarged, swollen and tender lymph nodes
• swollen & painful joints
• tender reddish bumps
• hoarse voice
• fever
• weight loss
• fatigue
• cough
• dyspnoea
• wheeze

Question 9

more specifically, what are the symptoms of acute sarcoidosis?

Answer 9

1) erythema nodosum
- type of skin inflammation
= reddish, painful, tender lumps

2) bilateral hilar lymphadenopathy
= enlarged, swollen and tender lymph nodes on the pulmonary Hilary

3) arthritis
= aching joints

4) uveitis
= inflammation of anterior chain of eye

5) fever

Question 10

more specifically, what are the symptoms of chronic sarcoidosis?

Answer 10

1) lung infiltrates
2) skin infiltrates
3) peripheral lymphadenopathy
4) hypercalcaemia

Question 11

many people present with a cough, exertional breathlessness and a vague chest discomfort. Symptomatic individuals can have a clear auscultation and may have a wheeze.
Describe what helps you make a prognosis of pulmonary sarcoidosis?

Answer 11

4 RADIOLOGICAL STAGES of lung involvement

Question 12

describe the 4 radiological stages of pulmonary sarcoidosis.

Answer 12

STAGE 1
= bilateral hilar lymphadenopathy

STAGE 2
= Pulmonary infiltrates WITH bilateral hilar lymphadenopathy

STAGE 3
= pulmonary infiltrates WITHOU bilateral hilar lymphadenopathy

STAGE 4
= fibrosis

Question 13

what are the characteristics of bilateral hilar lymphadenopathy?

Answer 13

• usually asymptomatic
• only detected by a chest X-ray
• occasionally, it is associated with headache, malaise & mild fever

Question 14

what is bilateral hilar lymphadenopathy?

Answer 14

bilateral enlargement of the lymph nodes of the pulmonary hila

• the hilum is the wedged shaped area on the centre of each lung

Question 15

name 7 extra pulmonary manifestations of sarcoidosis.

Answer 15

1) skin lesions
2) eye lesions
3) metabolic manifestations
4) central nervous system
5) bone & joint movement
6) hepatosplenomegaly
7) cardiac involvement

Question 16

describe the most common skin lesion likely to occur as a result of sacoidos?

Answer 16

erythema nodosum

Question 17

what is erythema nodosum?

Answer 17

type a skin inflammation causing reddish, lumps usually in the lower limbs, affecting most commonly the front of your knee

Question 18

what is the most common eye lesion likely to occur in sarcoidosis?

Answer 18

Uveitis

Question 19

what are the 2 kinds of uveitis?

Answer 19

1) anterior uveitis

2) posterior uveitis

Question 20

what does ANTERIOR uveitis present with?

Answer 20

• misting of vision

- painful, RED EYE

Question 21

what does POSTERIOR uveitis present with?

Answer 21

• progressive loss of vision.

Question 22

what is hepatosplenomegaly?

Answer 22

when the liver and spleen swell beyond there normal size, usually due to an infection

Question 23

what 8 investigations could be done to diagnose sarcoidosis?

Answer 23

1) imaging
2) full blood count
3) biochemistry
4) serum angiotensin converting enzyme
5) bronchoalveolar lavage
6) transbronchial biopsy
7) endobronchial biopsy
8) lung function tests

Question 24

what two modes of imaging are used?

Answer 24

1) chest X-ray

2) High resolution CT scan

Question 25

what can a CT scan show?

Answer 25

• shows nodules
• aggregation of nodules into larger nodules or masses
• reticulation
• honeycombing

Question 26

what two important chemicals are raised in sarcoidosis?

Answer 26

1) Calcium

3) angiotensin converting enzyme

Question 27

what are 2 ways that calcium is increased?

what do they mean?

Answer 27

1) hyerpcalcaemia
= increased calcium levels in blood

2) hypercalciuria
= increased calcium levels in the urine

Question 28

describe why increased levels of angiotensin converting enzyme is not used as a diagnostic test but as a marker test?

Answer 28

because the levels of ACE are increased in many other diseases, not just sarcoidosis

Question 29

what typically would a bronchoalveolar lavage test show?

Answer 29

• it would show lymphocytosis

- raised CD4:CD8 ratio

Question 30

what sort of lung defect would a lung function test show?

Answer 30

it would show restrictive lung defects

& reduced gas transfer

Question 31

how would you commonly treat sarcoidosis?

Answer 31

• corticosteroids
  = they improve the radiological appearance
  = but don’t have much of an affect on lung function tests

Question 32

what is the first line treatment, i.e. what corticosteroid drug is used?

Answer 32

prednisolone

Question 33

what immunosuppressants could be given?

Answer 33

• methotrexate
• azathioprine
• anti-TNF therapy
• hydroxycholorquine