Sarcoidosis

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

Prognosis: 2/3 remit within a decade, 1/3 progressive


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

Grutters, J. and van der Bosch, J. Corticosteroid treatment in sarcoidosis. Eur Respir J. 2006; 28(3):627-636.

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.

Wu, J. and Schiff, K. Sarcoidosis. Am Fam Physician. 2004; 70(2):312-322.