Sarcoidosis Flashcards
What is sarcoidosis
Multi-system granulomatous disorder of unknown cause associated with HLA-DRB1 and DQB1
Sarcoidosis presentation
Often asymptomatic Acute presents with: Fever Erythema nodosum BHL Polyarthralgia
Dry cough
Progressive dyspnoea/dec exercise tolerance
Chest pain
Lots of extra pulmonary signs
Sarcoidosis investigations
Raised ESR, LFTs and Ca
CXR shows BHL, pulmonary infiltrates and honeycombing depending on stage
XR shows punched out lesions on terminal phalanges
Tissue biopsy diagnostic with non-caseating granulomas
Lung FTs may be normal or show reduced lung volumes and a restrictive ventilatory defect
Sarcoidosis staging
CXR staging
0 - normal
1 - BHL
2 - BHL + peripheral pulmonary infiltrates
3 - peripheral pulmonary infiltrates alone
4 - progressive pulmonary fibrosis, bulla formation (honey combing), pleural involvement
Sarcoidosis management stage 1 or 0
Bed rest
NSAIDs
Spontaneously recover
Sarcoidosis management corticosteroid indications
Pred 40mg/d PO 4-6wks
Parenchymal lung disease
Uveitis
Hypercalcaemia
Neuro/cardio involvement
Sarcoidosis management stage 2+ (severe illness)
IV methylprednisolone
Immunosuppressants
Anti-TNF-alpha
Lung transplant
Sarcoidosis prognosis
60% with thoracic resolve over 2yrs
20% respond to steroids
In the rest improvement unlikely despite therapy