Granulomatous lung diseases - SARCOIDOSIS Flashcards
What is a granuloma?
& how is a granuloma formed?
= a mass or nodule composed of chronically inflamed tissue
A granuloma is formed by;
= the response of mononuclear phagocyte system
why do granulomas form?
they form to confine a pathogen and limit the extent of surrounding inflammation and tissue destruction
what is sarcoidosis?
a chronic disease caused by the ENLARGEMENT OF LYMPH NODES in many parts of the body and causes the appearance of NON-CASEATING GRANULOMATOUS.
where in the body does sarcoidosis have an affect?
- most commonly just the lungs
- but it is has multi-system involvement;
e. g. lymph nodes, joints, liver, skin, eyes, kidney, brain, nerve, heart
how important is smoking when looking at sarcoidosis?
sarcoidosis is less common in smokers.
what causes sarcoidosis?
- The cause is unknown.
However, there are a number of possibilities;
- e.g. imbalance of immune system with Type 4 hypersensitivity
What are the 2 types of sarcoidosis?
1) acute sarcoidosis
2) chronic sarcoidosis
what are the symptoms of sarcoidosis?
- enlarged, swollen and tender lymph nodes
- swollen & painful joints
- tender reddish bumps
- hoarse voice
- fever
- weight loss
- fatigue
- cough
- dyspnoea
- wheeze
more specifically, what are the symptoms of acute sarcoidosis?
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
more specifically, what are the symptoms of chronic sarcoidosis?
1) lung infiltrates
2) skin infiltrates
3) peripheral lymphadenopathy
4) hypercalcaemia
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?
4 RADIOLOGICAL STAGES of lung involvement
describe the 4 radiological stages of pulmonary sarcoidosis.
STAGE 1
= bilateral hilar lymphadenopathy
STAGE 2
= Pulmonary infiltrates WITH bilateral hilar lymphadenopathy
STAGE 3
= pulmonary infiltrates WITHOU bilateral hilar lymphadenopathy
STAGE 4
= fibrosis
what are the characteristics of bilateral hilar lymphadenopathy?
- usually asymptomatic
- only detected by a chest X-ray
- occasionally, it is associated with headache, malaise & mild fever
what is bilateral hilar lymphadenopathy?
bilateral enlargement of the lymph nodes of the pulmonary hila
- the hilum is the wedged shaped area on the centre of each lung
name 7 extra pulmonary manifestations of sarcoidosis.
1) skin lesions
2) eye lesions
3) metabolic manifestations
4) central nervous system
5) bone & joint movement
6) hepatosplenomegaly
7) cardiac involvement
describe the most common skin lesion likely to occur as a result of sacoidos?
erythema nodosum
what is erythema nodosum?
type a skin inflammation causing reddish, lumps usually in the lower limbs, affecting most commonly the front of your knee
what is the most common eye lesion likely to occur in sarcoidosis?
Uveitis
what are the 2 kinds of uveitis?
1) anterior uveitis
2) posterior uveitis
what does ANTERIOR uveitis present with?
- misting of vision
- painful, RED EYE
what does POSTERIOR uveitis present with?
- progressive loss of vision.
what is hepatosplenomegaly?
when the liver and spleen swell beyond there normal size, usually due to an infection
what 8 investigations could be done to diagnose sarcoidosis?
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
what two modes of imaging are used?
1) chest X-ray
2) High resolution CT scan
what can a CT scan show?
- shows nodules
- aggregation of nodules into larger nodules or masses
- reticulation
- honeycombing
what two important chemicals are raised in sarcoidosis?
1) Calcium
3) angiotensin converting enzyme
what are 2 ways that calcium is increased?
what do they mean?
1) hyerpcalcaemia
= increased calcium levels in blood
2) hypercalciuria
= increased calcium levels in the urine
describe why increased levels of angiotensin converting enzyme is not used as a diagnostic test but as a marker test?
because the levels of ACE are increased in many other diseases, not just sarcoidosis
what typically would a bronchoalveolar lavage test show?
- it would show lymphocytosis
- raised CD4:CD8 ratio
what sort of lung defect would a lung function test show?
it would show restrictive lung defects
& reduced gas transfer
how would you commonly treat sarcoidosis?
- corticosteroids
= they improve the radiological appearance
= but don’t have much of an affect on lung function tests
what is the first line treatment, i.e. what corticosteroid drug is used?
prednisolone
what immunosuppressants could be given?
- methotrexate
- azathioprine
- anti-TNF therapy
- hydroxycholorquine
