Sarcoidosis
Description
- Multisystem granulomatous disease of unknown cause
- Defining lesion: non-caseating (non-necrotising) epithelioid cell granuloma
- *A diagnosis of exclusion - compatible clinical picture + granulomas + no alternative cause*
Organ involvement
| Organ | Frequency | Presentation |
|---|---|---|
| Lung | 89-99% | Cough, dyspnoea, wheeze, stridor |
| Skin | 16-32% | Lupus pernio, nodules, plaques, infiltration of old scars and tattoos |
| Eye | 5-23% | Painful red eye, uveitis |
| Liver | 12-20% | Deranged LFTs (cholestatic), abdominal pain |
| Lymph nodes | 13-15% | Peripheral lymphadenopathy |
| Spleen | 5-10% | Splenomegaly, abdominal pain, cytopenias |
| Nervous system | 3-9% | Facial palsy, headache, gait disturbance, hearing loss, paraesthesia |
| Heart | 2-5% clinically (~25% at autopsy) | Conduction block, VT, heart failure, syncope, sudden death |
Named syndromes
- Lofgren syndrome - erythema nodosum + bilateral hilar lymphadenopathy + fever + arthralgia (typically ankles)
- *Acute, self-limiting, >80-90% spontaneous resolution - a good-prognosis presentation that needs no biopsy*
- Heerfordt syndrome (uveoparotid fever) - uveitis + parotid enlargement + fever + facial nerve palsy
- Blau syndrome - genetic (NOD2) granulomatous disease of childhood; not sarcoidosis
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