Sarcoidosis is a multi-system granulomatous disorder of unknown cause.
It is asymptomatic in up to 40% of cases, being discovered incidentally on a routine CXR.
Features of sarcoidosis may be divided into pulmonary and non-pulmonary:
Pulmonary features of sarcoidosis:
- dry cough
- progressive dyspnoea
- chest pain
- wheeze
Non-pulmonary features of sarcoidosis include:
- Lymphadenopathy
- Skin involvement (25%). Includes erythema nodosum (affects 1 in 10) and lupus pernio (which affects more females)
- Liver involvement
- Occular involvement – anterior uveitis, glaucoma, cataract
- Polyarthralgia
- Neurosarcoid – including Bells palsy
- Hypercalcaemia (10%). Due to dysregulated production of calcitriol by activated macrophages and granuloma
- Cardiac involvement
- Renal stones
90% of patients with sarcoid will have an abnormal CXR.
Staging of CXR in sarcoid is:
- 0 = clear CXR
- 1 = bilateral hilar lymphadenopathy (BHL)
- 2 = bilateral hilar lymphadenopathy plus pulmonary infiltration
- 3 = pulmonary infiltration only
- 4 = fibrosis, honeycombing, pleural involvement
Note staging is purely radiological and does not correlate with pulmonary function
Investigations:
- CXR
- HRCT
- Bloods
- Raised serum ACE (50%)
- Raised calcium (10%)
- LFTs - looking for liver involvement
- Possibly lymphopenia
- Urinary calcium
- Biopsy - TBLB (transbronchial lung biopsy) and endobronchial
- Lung function tests
- ECG - ?cardiac involvement
Management:
- BHL alone doesn't need treatment, neither does stage II or III disease if lung function tests are only mildly abnormal
- acute sarcoidosis : NSAIDs and bed rest
- prednisolone ( 0.5/mg/kg for 4 weeks then reducing dose to control symptoms for 12-24 months) is indicated if:
- symptomatic pulmonary disease or progressive loss of lung function
- cardiac disease
- neurological disease
- eye disease
- symptomatic hypercalcaemia
Methotrexate decreases requirement for steroid.
Bisphosphonates should be given to protect bone.
In end
Bisphosphonates should be given to protect bone.
In end
Prognosis: 2/3 remit within a decade, 1/3 progressive
Last reviewed: Jan 2015
Last reviewed: Jan 2015
Small print gem: Löfgren's syndrome is
a form of sarcoidosis consisting of arthritis, erythema nodosum, and
bilateral hilar lymphadenopathy. Females more affected than males. Good
prognosis – most cases resolve.
References
Iannuzzi, M., Rybicki, B. and Teristein, A. Sarcoidosis. N Engl J Med 2007; 357(21):2153-2165.
Wells, A. et al. Intersitial lung disease guideline: the British Thoracic Society in collaboration with the Thoracic Society of Australia and New Zealand and the Irish Thoracic Society. Thorax. 2008; 63;v1-v58.
Wells, A. et al. Intersitial lung disease guideline: the British Thoracic Society in collaboration with the Thoracic Society of Australia and New Zealand and the Irish Thoracic Society. Thorax. 2008; 63;v1-v58.
