Sarcoidosis

Question 1

Define and describe epidemiology of sarcoidosis

Answer 1

Multisystem non-caseous granulomatous inflammatory disorder - diagnosis of exclusion
Most common in africans/scandinavians, peaking in 3rd and 5th decade

Question 2

What does sarcoidosis commonly affect and what is the pathology?

Answer 2

Lungs, skin and eyes affected

1. Unknown antigen presented MHC class 2 complexes on macrophages to CD4+ T-lymphocytes
2. Accumulate and release cytokines
3. Formation of non-caseating granulomas in organs

Question 3

What are RFs for sarcoidosis?

Answer 3

1. FHx sarcoidosis - increases risk 4x
2. Female
3. Non-smokers

Question 4

What are general symptoms of sarcoidosis?

Answer 4

Fever
Malaise
Weight loss
Bilateral parotid swelling (parotitis) - other causes of this are Sjorgen’s, alchohol and mumps

Question 5

What are pulmonary symptoms of sarcoidosis?

Answer 5

Bilateral hilar lymphadenopathy
Breathlessness
Dry cough
Chest discomfort
Minimal clinical signs

Question 6

What are clinical signs of sarcoidosis?

Answer 6

Skin: lupus pernio, erythema nodosum
CNS: meningitis, cranial nerve lesions
Eyes: Uveitis, keratoconjunctivitis
Parotids: bilateral enlargment
Lungs: BHL, fibrosis, lymphocytosis
Liver: Hepatitis, cholestasis and cirrhosis

Question 7

What are the CXR stages?

Answer 7

Stage 0: Normal
Stage 1: BHL
Stage 2: BHL + pulmonary infiltrates
Stage 3: Pulmonary infiltrates without hilar lymphadenopathy
Stage 4: Extensive fibrosis with distortion

Question 8

What would other general investigations show?

Answer 8

FBC - anaemia, leukopenia
U&Es - hypercalcaemia + hypercalciuria (vitamin D hydroxylation in macrophages in granulomas)
Serum urea - elevated
Creatinine - elevated
High serum ACE

Question 9

What signs are seen on a CT chest?

Answer 9

Ground glass = reversible
Cystic architectural distortion = irreversible
Check for lymph node involvement

Question 10

What would a bronchoscopy with USS guided biopsy of hilar lymphadenopathy refelect?

Answer 10

Non-caseating granulomas with epitheliod cells (activated macrophages), multinucleate Langerhans cells and mononuclear cells (lymphocytes).

Question 11

How is sarcoidosis managed?

Answer 11

1. No treatment if no symptoms
2. Corticosteroids if patient has uveitis, hypercalcaemia, parenchymal lung disease and neurological/cardiac involvement - high oral doses initially (40mg/24hrs) for 4-6 weeks then wean till 1 year with dose increases for flare-ups
3. Bone protection if medicating for long - bisphosphonates
4. 2nd line - Immunosuppressive agents (methotrexate)

Opthalmology review to check for permanent eye damage

Question 12

What factors indicate poorer prognosis?

Answer 12

1. Black ancestry
2. Chronic pulmonary involvement
3. Lupus pernio
4. Chronic hypercalcaemia