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RESPIRATORY > SARCOIDOSIS > Flashcards

SARCOIDOSIS Flashcards

1
Q

What is sarcoidosis?

A

a multisystem disorder of unknown aetiology characterised by non-caseating granulomas (nodules of inflammation full of macrophages)

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2
Q

Which groups of people is sarcoidosis more common in?

A

Young adults and age 60
Women are more often affected
Occurs more frequently in black people, particularly of African descent

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3
Q

What are features of sarcoidosis?

A

Most commonly:

Lungs - progressive dyspnoea, productive cough
Systemic - swinging fever, fatigue, weight loss
Eyes - uveitis, glaucoma
Skin - erythema nodosum, lupus pernio, granulomas in scar tissue, papular sarcoidosis

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4
Q

Why does sarcoidosis cause hypercalcaemia?

A

macrophages inside the granulomas cause an increased conversion of vitamin D to its active form (1,25-dihydroxycholecalciferol)

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5
Q

What is Lofgren’s syndrome?

A

an acute form of sarcoidosis characterised by a triad of:
bilateral hilar lymphadenopathy (BHL)
erythema nodosum
polyarthralgia

It usually carries an excellent prognosis

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6
Q

What is Heerfordt’s syndrome?

A

AKA uveoparotid fever
Characterised by parotid enlargement, fever, facial nerve palsy and uveitis secondary to sarcoidosis

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7
Q

What is Mikulicz syndrome?

A

Aka Sjögren’s syndrome

Enlargement of parotid and lacrimal glands due to sarcoidosis, TB or lymphoma

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8
Q

How do we investigate sarcodiosis?

A

Raised serum ACE levels
Bone profile - Hypercalcaemia
raised ESR/CRP
Raised serum soluble IL-2 receptor
Raised immunoglobulins
U&Es for kidney involvement
Urine dipstick for ACR and proteinuria - nephritis
LFTs for liver involvement
Ophthalmology
ECG and echo - heart involvement
USS abdo for liver and kidney involvement
May do Mantoux test to rule out TB

CXR and CT thorax
MRI for CNS involvement
PET scan can show active inflammation in affected areas

Spirometry - restrictive
USS guided biopsy of mediastinal lymph nodes- non-caseating granulomas with epithelioid cells

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9
Q

What may you see on CXR?

A

Fibrosis
Bilateral hilar lymphadenopathy

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10
Q

What organs does sarcoidosis affect?

A

Lungs >90%
Liver 20%
Eyes 20%
Skin 15%
Heart 5%
Kidneys 5%
CNS 5%
PNS 5%
Bones 2%

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11
Q

How does sarcoidosis affect the lungs?

A

Causes pulmonary fibrosis, nodules and mediastinal lymphadenopathy

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12
Q

How does sarcoidosis affect the liver?

A

Causes liver nodules, cirrhosis, cholestasis

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13
Q

How does sarcoidosis affect the eyes?

A

Can cause uveitis, conjunctivitis, optic neuritis
May cause

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14
Q

How does sarcoidosis affect the skin?

A

Can cause erythema nodosum, lupus pernio and granulomas within scar tissues

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15
Q

What is erythema nodosum?

A
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16
Q

What is lupus pernio?

A
17
Q

How does sarcoidosis affect the heart?

A

Can cause BBB, heart block and myocardial muscle involevemtn

18
Q

How does sarcoidosis affect the kidneys?

A

Can cause kidney stones due to hypercalcaemia
Can also cause nephrocalcinosis and interstitial nephritis

19
Q

How does sarcoidosis affect the CNS?

A

It can cause nodules, pituitary involvement e.g. diabetes insipidus and encephalopathy

20
Q

How does sarcoidosis affect the PNS?

A

Can cause facial nerve palsy or mono neuritis multiplex

21
Q

How does sarcoidosis affect the bones?

A

Can cause arthralgia, arthritis and myopathy

22
Q

Why is serum ACE often elevated in sarcoidosis?

A

Epithelioid cells in granulomas produce ACE

23
Q

What staging system can be used for Sarcoidosos?

A

Scadding staging

24
Q

Whats the prognosis of sarcoidosis?

A

Spontaneously revolves within 6 months in around 60% of patients
In a small number of patients it progresses with pulmonary fibrosis and pulmonary hypertension, potentially requiring a lung transplant.
Death in sarcoidosis is usually when it affects the heart (causing arrhythmias) or the central nervous system.

25
Q

How is sarcoidosos managed?

A

No treatment if no symptoms/mild symptoms
Oral steroids for 6-24 months if needed (with bisphosphonates to protect against osteoporosis)
Lung transplant

26
Q

What are indications for steroids to manage sarcoidosis?

A

patients with chest x-ray stage 2 or 3 disease who are symptomatic. Patients with asymptomatic and stable stage 2 or 3 disease who have only mildly abnormal lung function do not require treatment
hypercalcaemia
eye, heart or neuro involvement

27
Q

What are second line agents for sarcoidosis?

A

Immunosuppressants e.g. methotrexate, azathioprine

28
Q

Outline the staging for CXR findings in sarcoidosis?

A

Stage 1 - bilateral hilar lymphadenopathy
Stage 2 - BHL with peripheral infiltrates
Stage 3 - peripheral infiltrates along
Stage 4 - pulmonary fibrosis

RESPIRATORY (22 decks)

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