Sarcoidosis

Question 1

What is sarcoidosis?

Answer 1

multisystem chronic inflammatory condition characterised by formation of non-caseating epithelioid granulomata

Question 2

What are non-caseating granulomas?

Answer 2

Non-necrotising nodules of inflammation + scarring

Question 3

In order of predilection, which organs are effected by sarcoidosis ?

Answer 3

Lungs (up to 90% cases)
Skin (up to 30% cases)
Eyes (up to 30% cases)
Brain, Nervous system, Live, Heart

Question 4

In which patient groups is sarcoidosis more common?

Answer 4

Young adults
People of African descent

Question 5

Give 4 acute features of sarcoidosis

Answer 5

Erythema nodosum
Bilateral hilar lymphadenopathy
Swinging fever
Polyarthralgia

Question 6

Give 4 insidious features of sarcoidosis

Answer 6

Dyspnoea
Non-productive cough
Malaise
Weight loss

Question 7

List 4 pulmonary symptoms seen in sarcoidosis

Answer 7

Dyspnoea
Dry persistent cough
Wheezing
Non-cardiac chest pain (tightness/ pleuritic discomfort)

Question 8

List 3 skin manifestations of sarcoidosis

Answer 8

Hyper-/ hypopigmentation
Erythema nodosum
Lupus pernio

Question 9

What is Lupus pernio?

Answer 9

Bluish-red/ violaceous nodules + plaques over nose, cheeks + ears

Question 10

What is the most common ocular manifestation of sarcoidosis?

Answer 10

Uveitis

Question 11

List 3 cardiac manifestations of sarcoidosis

Answer 11

Arrhythmia
Congestive heart failure
Syncope

Question 12

What electrolyte abnormality if characteristic of sarcoidosis? Why?

Answer 12

Hypercalcaemia
Macrophages in the granulomas cause conversion of vitamin D to its active form

Question 13

3 features in bloods in sarcoidosis

Answer 13

Raised serum ACE
Hypercalcaemia
Raised ESR
Raised ALP

Question 14

What is the use of ACE in diagnosis of sarcoidosis?

Answer 14

60% sensitivity
70% specificity
Not reliable in dx, but have a role in monitoring disease activity

Question 15

What may be found in 24 hr urine collection in sarcoidosis?

Answer 15

Hypercalciuria

Question 16

Describe the staging of sarcoidosis based on CXR findings

Answer 16

Stage 0: normal
Stage 1: bilateral hilar lymphadenopathy
Stage 2: BHL + interstitial infiltrates
Stage 3: diffuse interstitial infiltrates, no BHL
Stage 4: diffuse fibrosis

Question 17

What can high resolution CT scanning show in sarcoidosis?

Answer 17

Interstitial lung disease

Question 18

What may spirometry show in sarcoidosis?

Answer 18

Restrictive defect

Question 19

What is seen on transbronchial/ lymph node biopsy in sarcoidosis?

Answer 19

Non-caseating granulomas consisting of:
Epithelioid cells (activated macrophages)
Multinucleate Langerhans cells
Mononuclear cells (lymphocytes)

Question 20

What is the course of disease in 2/3 of patients with sarcoidosis?

Answer 20

Remits without Tx in ~2/3

Question 21

What are the indications for corticosteroid treatment for sarcoidosis?

Answer 21

CXR stage 2-3 + symptomatic
Uveitis (Prednisolone PO)
Hypercalcaemia
Neurological/ cardiac involvement

Question 22

Name 1 protective factor against sarcoidosis

Answer 22

Cigarette smoking

Question 23

List 5 markers of poor prognosis in sarcoidosis

Answer 23

Insidious onset, Sx >6 months
Absence of erythema nodosum
Extrapulmonary manifestations e.g. lupus pernio, splenomegaly
CXR: stage III-IV features
Black African or Afro-Caribean ethnicity

Question 24

List 3 syndromes associated with sarcoidosis

Answer 24

Lofgren’s syndrome
Mikulicz syndrome
Heerfordt’s syndrome

Question 25

What is Lofgren’s syndrome?

Answer 25

Acute form of sarcoidosis characterised by:
Bilateral hilar lymphadenopathy
Erythema nodosum
Fever
Polyarthralgia

Question 26

What is Mikulicz syndrome?

Answer 26

Enlargement of parotid + lacrimal glads due to sarcoidosis, TB or lymphoma

Now considered outdated as confusing overlap with Sjogren’s syndrome

Question 27

What is Heerfordt’s syndrome?

Answer 27

aka Uveoparotid fever
Parotid enlargement + uveitis secondary to sarcoidosis