sarcoidosis

Question 1

definiton of sarcoidosis

Answer 1

Multisystem granulomatous inflammatory disorder.

Question 2

aetiology of sarcoidosis

Answer 2

unknown

transmissible agents (e.g. viruses, atypical mycobacterium, Propionibacterium acnes)

env triggers - HLA-DRB1 and DQB1 alleles

genetic factors

pathology

• The unknown antigen is presented on the MHC Class II complex of macrophages to CD4 (Th1) lymphocytes, which accumulate and release cytokines (e.g. IL-1/IL-2)
• = non-caseating granulomas in a variety of organs

Question 3

epidemiology of sarcoidosis

Answer 3

uncommon

20-40yr

africans - more commonly and more severely, especially by extra-thoracic disease

females

prevalence is variable ww - highest in N europe

.Prevalence in UK is 16 in 100000 (highest in Irish women)

Question 4

general sx of sarcoidosis

Answer 4

• asx
• fever
• malaise
• weight loss
• bilateral parotid swelling
• lymphadenopathy
• splenomegaly
• hepatosplenomegaly
• enlargement of lacrimal and parotid glands
• hypercalcaemia
• hypercalciuria
• renal stones

Question 5

lung sx of sarcoid

Answer 5

progressive breathlessness

cough - unproductive

chest discomfort

reduced exercise tolerance

fine inspiratory crepitations

abnormal XR with bilateral huler lymphadenopathy +- pul infiltrates or fibrosis

Question 6

MSK sx of sarcoid

Answer 6

bone cysts (eg dactylitis in phalanges)

polyarthralgia

myopathy

Question 7

eye sx of sarcoid

Answer 7

keratoconjunctivitis sicca (dry eyes)

uveitis

papilloedema

conjunctivitis

glaucoma

Question 8

skin signs of sarcoid

Answer 8

lupus pernio (red–blue infiltrations of nose, cheek, ears, terminal phalanges),

erythema nodosum,

maculopapular eruptions.

subcutaneous nodules

Question 9

neuro sx of sarcodi

Answer 9

• lymphocytic meningitis
• space-occupying lesions
• pituitary infiltration
• cerebellar ataxia
• cranial nerve palsies (eg bilateral facial nerve palsy)
• bell’s palsy
• neuropathy
• brainstem and spinal syndromes

Question 10

cardio sx of sarcoid

Answer 10

arrhythmia

BBB

paricarditis

cardiomyopathy

congestive cardiac failure

Question 11

acute sarcoidosis

Answer 11

fever

erythema nodosum

polyarthralgia

bilateral hilar lymphadenopathy also called Löfgren syndrome, which usually resolves spontaneously.

Question 12

Ix for sarcoid

Answer 12

blood

24hr urine collection - hypercalciuria

CXR

high resolution CT - for diffuse lung involvement

67Gallium scan:- Shows areas of inflammation (classically parotids and around eyes)

pulmonary function tests - reduced FEV, FVC and gas transfer (restrictive picture)

bronchoscopy and bronchoalveolar lavage

Transbronchial lung biopsy (or lymph node biopsy)

ECG - arrhythmia, BBB

US - nephrocalcinosis or hepatosplenomegaly

bone XR - punched out lesions in terminal phalanges

CT/MRI - assess severity of pul disease or diagnosis neurosarcoidosis

Ophthalmology assessment (slit lamp examination, fluorescein angiography) is indicated in ocular disease.

Question 13

blood for sarcoid

Answer 13

raised serum ACE

raised Ca

raised ESR

FBC (WCC may be low because of lymphocyte sequestration in lungs - lymphopenia)

raised immunoglobins (polyclonal hyperglobulinaemia)

LFT (Raised alkaline phosphatase and GGT)

Question 14

CXR for sarcoid

Answer 14

stage 0 - may be clear

stage 1 - bilateral hilar lymphadenopathy

stage 2 - stage 1 with pul infiltration and paratracheal node enlargement

stage 3 - pul infiltration and fibrosis, bulla formation (honey-combing), pleural involvement

Question 15

broncoscopy and BAL for sarcoid

Answer 15

raised lymphocytes with raised CD4:CD8 ratio in active disease

high neutrophils with pul fibrosis

Question 16

transbronchial lung biopsy/LN biopsy for sarcoid

Answer 16

Non-caseating granulomas

composed of epithelioid cells (activated macrophages), multinucleate Langhans cells and mononuclear cells (lymphocytes).

Question 17

mx of sarcoidosis

Answer 17

acute resp failure:
* IV or oral steroid (methylpred IV, pred oral)
* ventilation
* oxygen

Question 18

mx of sarcoid pul disease

Answer 18

1. obstervation
2. oral/inhaled steroid
3. immunosuppression - methotrexate/Azathiorprine
4. oxygen
5. infliximab
6. lung transplant

Question 19

mx of cutaneous disease in sarcoid

Answer 19

erythematous scaly rash and/or papules
* topical steroid
* oral steroid
* immunosuppression - hydroxychloroquine/methotrexate
* infliximab

lupus pernio with scarring
* oral steroid
* immunosuppressive agent
* infliximab

Question 20

mx of sarcoid with CNS or PNS involvement

Answer 20

1. oral steroid
2. immunosuppression - methotrexate
3. infliximab

Question 21

mx of sarcoid renal disease

Answer 21

hypercalcaemia/hypercalciuria
* oral steroid
* hydroxychloroquine / ketoconazole

granulomatous interstitial nephritis
* oral steroid
* immunosuppression - azathioprine / mycophenolate mofetil
* infliximab

Question 22

sarcoidosis monitoring

Answer 22

every 3-6mo
clinical signs of disease activity
pul functuion tests, CXR
FBC
calcium - risk of hypercalcaemia
LFTs - adverse effects from immunosuppression
bone density screening

Question 23

complications of sarcoidosis

Answer 23

severe or life threatening haemoptysis
steroid related SE
pul arterial HTN
glaucoma
immunosuppressant toxicity - hepatic and bone marrow
congesitive heart failure
meningeal disease
CNS parenchymal disease
spinal canal disease
neuropathy
myopathy
cataract
vascular retinitis
optic atrophy
vision loss
hydroxychloroquine-related renal toxicity
pit lesions
infection
hypercalcaemia
heart block
ventricular arrhythmias
cranial neuropathies

Question 24

Px of sarcoidosis

Answer 24

good especially if just lung