Sarcoidosis | Wegener granulomatosis | |
Clinical presentation | ||
Upper airway | Occurs in <10% of cases | Extremely common |
Lung | Common Adenopathy Diffuse infiltrates, usually upper lobes | Common Nodular infiltrates, sometimes with cavities Infiltrates localised to areas of haemorrhage, often lower lobe |
Eye | Uveitis, retinitis, optic neuritis | Episcleritis |
Skin | Maculopapular lesions | Vasculitic lesions |
Lupus pernio | Occasionally seen | |
Kidney | Glomerulopnephritis rare, renal failure usually due to hypercalcaemia | Focal necrotising glomerulitis in over half of cases |
Neurological disease | 10% of patients. Often mass seen on MRI | Up to 30% of cases will have lesions. Usually due to vasculitic lesions |
Joints | Uncommon. Can see specific bone cysts | Uncommon |
Tracheal/proximal airway stenosis | Rare | Common |
ANCA | Negative | Positive in >80% of cases |
ACE | Positive in >60% of new cases | Negative |
Treatment | ||
Corticosteroids | Drug of choice for initial management | Supportive but not used for maintenance therapy |
Methotrexate | Effective | Effective |
Azathioprine | Effective | Effective |
Cyclophosphamide | Reserved for refractory cases | Treatment for extensive disease |
Anti-TNF therapy | Effective for refractory disease | Not effective for most patients |
Ritixumab | Effectiveness unknown | Effective for refractory cases |
ANCA, antineutrophil cytoplasmic antibody; TNF, tumour necrosis factor.