- A multisystem granulomatous disorder presenting usually as
- Bilateral hilar lymphadenopathy
- Pulmonary infiltration
- Skin/eye lesions
Epidemiology
- 19 in 100000
- Female > Male
- More severe in Blacks than Whites
Aetiology
- Unknown
Clinical features
- Third or fourth decade
Extrapulmonary features
- Skin
- Erythema nodosum
- Lupus pernio
- Eye
- Uveitis
- Conjunctivitis
- Keratoconjunctivitis sicca
- Face
- Parotitis
- Facial nerve palsy
- Metabolic
- Hypercalcaemia (10%)
- CNS
- Meningoencephalitis
- Spinal cord disease
- Myopathy
- Polyneuropathy
- Gastrointestinal
- Hepatosplenomegaly
- Cardiovascular
- Cardiomyopathy
Investigations
- Chest X-ray
- CT chest
- Blood tests
- FBC (normocytic anaemia)
- Increased ESR
- Increased Ca++
- Increased Serum angiotensin-converting enzyme (ACE)
- Transbronchial biopsy
- Spirometry
- Restrictive defect
- Decreased Gas transfer
Presentations
- Respiratory symptoms / abnormal chest X-ray
- 50%
- Fatigue or weight loss
- 5%
- Peripheral lymphadenopathy
- 5%
- Fever
- 4%
- Normal chest X-ray
- 20%
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