Expansion
Non-caseating granulomas affecting any organ, most often the lung
Expansion
Chest radiograph staging
- Stage 0 - normal
- Stage 1 - bilateral hilar lymphadenopathy alone
- Stage 2 - lymphadenopathy plus pulmonary infiltrates
- Stage 3 - infiltrates without lymphadenopathy
- Stage 4 - fibrosis
“The stages march from nodes, to nodes plus lung, to lung alone, to scar.”
Extrapulmonary features
- Skin: erythema nodosum, lupus pernio (specific), scar infiltration
- Eyes: anterior uveitis, keratoconjunctivitis sicca
- Cardiac: arrhythmia, heart block, cardiomyopathy. A leading cause of death
- Neurological: facial nerve palsy, meningitis, pituitary involvement
- Metabolic: hypercalcaemia, from macrophage 1-alpha-hydroxylase activity
- Others: parotid enlargement, hepatosplenomegaly, arthralgia, bone cysts
Lofgren’s syndrome is an acute presentation with erythema nodosum, bilateral hilar lymphadenopathy, fever and polyarthralgia. It carries an excellent prognosis and usually resolves without treatment.
Investigations: raised serum ACE (neither sensitive nor specific), hypercalcaemia, CT, and non-caseating granulomas on biopsy.
Treatment is corticosteroids where there is significant organ involvement: progressive lung disease, cardiac or neurological involvement, hypercalcaemia or eye disease.