Definition: Multisystem disorder characterised by non-caseating
epithelioid cell granulomata.
Prevalence 27:100 000. M:F roughly equal, slighty more common in women.
More common among West Indians.
MHC class II macrophages present unknown antigen to TH1.
T-cells proliferate and release immune mediators. Macrophages are
recruited and transform into epitheliod cells and Langhan's giant cells.
langhan's cells may show cytoplasmic inclusions: calcium carbonate crystals,
Schaumann bodies, asteroid bodies.
Breathlessness, anterior uveitis, abnormal CXR, superficial lymphadenopathy.
Constitutional: fever, malaise, weightloss.
- erythema nodosum
- lupus pernio
- transient vesicular eruptions on fingers
- violaceous skin papules
- Cranial neuropathies
- Lymphocytic meningitis
- Diabetes insipidus
- Anterior uveitis
- Cor pulmonale
- Heart block
- Bilateral hilar lymphadenopathy
- Reticulonodular shadowing
- Nephrocalcinosis, calciuria
If barn door, then no histology required.
- spiral CT thorax
- transbronchial biopsy
- lung function tests
- previously psitive Heaf test may now be negative
Serial CXR, serum ACE levels.
Remits without treatment in 2/3 of whites, 1/3 of blacks. Fatal in <3%.
Steroids may help the more severe manifestations. May try chloroquine.
Azathioprine or methotrexate may be tried as steroid sparing agents.