Sarcoidosis

Question 1

Define sarcoidosis

Answer 1

Chronic granulomatous multisystem disorder, characterised by the accumulation of T lymphocytes and macrophages and non-caseating granuloma formation.

Question 2

Risk factors for sarcoidosis

Answer 2

20-40yrs
FHx sarcoidosis
Scandinavian origin
Female
Non-smoker
Black ancestry - Afro-Caribbeans
Agricultural exposure, insecticides

Question 3

What is acute sarcoidosis/Lofgren syndrome

Answer 3

Self-limiting distinct form where there is:
1. Hilar adenopathy
2. peri-arthritis (inflammation AROUND joints)
3. Erythema nodosum

Question 4

Symptoms of sarcoidosis

Answer 4

General: Fever, malaise, weight loss, parotid swelling, lymphadenopathy

Lungs (90%): SOB | dry cough | chest discomfort | wheeze
MSK: bone pain | arthralgia
Eyes: dry | swelling | pain | photophobia
Skin: erythema nodosum | maculopapular erosions | Lupus pernio (red-blue infiltrations of nose, cheek, terminal phalanges, ears)
Neuro: facial palsy | parotid gland enlargement | headache
Cardiac: chest pain | arrhythmia

Question 5

Signs of sarcoidosis on examination

Answer 5

General: Parotid swelling, lymphadenopathy, hepatosplenomegaly

Lungs: fine inspiratory crepitations
MSK: polyarthalgia | myopathy
Eyes: red (uveitis) | papilloedema
Skin: erythema nodosum | maculopapular erosions | Lupus pernio (red-blue infiltrations of nose, cheek, terminal phalanges, ears)
Neuro: CNVII palsy | peripheral neuropathy | cerebellar signs
Heart: arrhythmia | heart failure

Question 6

Investigations for sarcoidosis

Answer 6

ECG: ?arrhythmia, BBB, pericarditis
Eye: uveitis, retinal vascular changes, conjunctival nodules, lacrimal gland enlargement

ACE: raised
Bone profile: hypercalcaemia
ESR: raised
CRP: raised
FBC: raised white cells + Ig
U&Es: raised Ur/Cr (renal involvement)
LFTs: ALP/GGT raised

CXR: peri-hilar lymphadenopathy
Spirometry: restrictive (reduced FEV1 + FVC, normal ratio)
HRCT: Diffuse lung involvement
Transbronchial lung biopsy: Non-caseating granulomas
Bronchoalveolar lavage: BA: lymphocytosis
Gallium 67: Panda sign uptake patterns

Question 7

Management for sarcoidosis

Answer 7

Asymptomatic: observation

Symptomatic:
First line: Steroids e.g. Prednisolone
+ Osteoporosis prevention e.g. bisphosphonates (caution with vit D/calcium due to risk of hypercalcaemia)
Second line: Steroid-sparing agents e.g. methotrexate, azathioprine, leflunomide, mycophenolate, hydroxychloroquine

Question 8

Complications of sarcoidosis

Answer 8

Pulmonary: Haemoptysis | pulmonary hypertension
Cardiac: congestive heart failure | heart block | ventricular arrhythmias
Neuro: meningeal disease | CNS parenchymal disease | spinal canal disease | neuropathy | myopathy | pituitary lesions
Ocular: Glaucoma | cataracts | vascular retinitis | vision loss | optic atrophy
Metabolic: hypercalcaemia

Question 9

Prognosis for sarcoidosis

Answer 9

Spontaneous remissions occur in 55% to 90% of patients with stage I disease
Most burns out within 1-2 years
Some require treatment and go into remission
Some may develop chronic sarcoidosis and continue to suffer flare ups