Sarcoidosis

Question 1

Definition of sarcoidosis?

Answer 1

multisystem granulomatous disorder of unknown etiology
usually 20-40s, northern european, black>white. F>M
associated with HLA-DRB1 and DQD1 alleles

Question 2

Clinical Features of sarcoidosis?

Answer 2

Incidentally discovered in about half of cases on CXR with bilateral hilar lymphadenopathy and are asymptomatic
Acute presentation - Erythema nodosum, polyarthralgia, fever and BHL

Question 3

pulmonary disease of sarcoidosis

Answer 3

Pulmonary disease is present in about 90%
Bilateral hilar lymphadenopathy
Pulmonary infiltrates or fibrosis
Dry cough, progressive dysnpoea, reduced ET, CP
10-20% Sx progress with subsequent deterioration in lung function

Question 4

extra-pulmonary manifestations of sarcoidosis?

Answer 4

present in around 30% 
 lymphadenopathy 
 Hepato/Splenomegaly 
 EYE: Conjunctivitis, uveitis, glaucoma, 
 Enlarged lacrimal and parotid glands 
 Terminal phalangeal bone cysts 
 SKIN: E.Nodosum, Lupus Pernio, Subcut nodules 
 HEART: cardiomegaly, arrhythmias 
 HyperCa, renal stones 
 Neuro: Bell's palsy

Question 5

Investigations for sarcoidosis?

Answer 5

Blood: Raised ESR, LFTs, Serum ACE, Ca and Ig
Urine: raised Ca
CXR:
USS: nephrocalcinosis or hepatosplenomagaly
PFT: N or reduced volumes, impaired gas transfer, Restrictive pattern
Biopsy: non-caseating granulomata
BAL: raised lymphocytes in active disease
Bone XR: punched out lesions in terminal phalanges
CT/MRI:
Ophthalmology assessment:

Question 6

Management?

Answer 6

BHL alone - don’t need tx in most cases

ACUTE: Bed rest and NSAIDs

Indications for Steroids(20-40mg OD pred for 4-6/52 then taper down over 1 year): parenchymal lung disease, uveitis, hypercalcaemia, Neuro/renal/cardia involvement

Other:
IV methyl pred for severe
Methotrexate, hydrochloroquine

Question 7

monitoring of sarcoidosis?

Answer 7

ECG, PFT’s Renal function

Question 8

Prognosis of Sarcoidosis?

Answer 8

60% Thoracic sarcoidosis resolve over 2 years
20% respond to steroids
20% dont…