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Sarcoidosis 

Sarcoidosis

Chapter:
Sarcoidosis
Author(s):

Robert P. Baughman

and Elyse E. Lower

DOI:
10.1093/med/9780199204854.003.1812_update_001

Update:

Imaging—discussion of use of endobronchial ultrasound and PET scanning.

Treatment—use of the humanized monoclonal anti-TNF antibody, adalimumab, in refractory cases.

A relevant case history from Oxford Case Histories in Respiratory Medicine has been added to this chapter.

Updated on 30 May 2013. The previous version of this content can be found here.
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date: 26 March 2017

Sarcoidosis is a disease of unknown cause that is characterized by the presence of noncaseating granulomas in at least two organs. It can present in a wide variety of ways. Differential diagnosis is most commonly from tuberculosis or lymphoma.

Clinical features

Respiratory involvement—described in more than 90% of patients and staged according to the chest radiograph appearance: Stage 1, hilar adenopathy alone; stage 2, adenopathy and parenchymal disease; stage 3, parenchymal disease alone; and stage 4, fibrosis. Such staging predicts outcome (resolution in 2–3 years—stage 1, 90%; stage 3, 30%) but not the degree of extrapulmonary disease. Pulmonary function studies typically demonstrate a restrictive pattern.

Skin involvement—the second most commonly affected organ: manifestations include hyperpigmentation; hypopigmentation; keloid reaction; waxy, maculopapular lesions, which when present on face are called lupus pernio and diagnostic of sarcoidosis; erythema nodosum.

Other organ involvement: eye—uveitis and lacrimal glands; neurological—cranial nerves (especially VII), central nervous system (lymphocytic meningitis, hypothalamic involvement) and peripheral nerves; liver—abnormal liver function tests in 50%; hypercalcaemia/hypercalciuria; heart—involvement is rare but can be serious with arrhythmic death.

Acute vs chronic disease—acute disease (which lasts for <2 years) is associated with erythema nodosum, hilar adenopathy, anterior uveitis and cranial nerve VII paralysis. Chronic disease includes such manifestations as lupus pernio, stage 4 chest radiograph, posterior uveitis, urolithiasis, and bone cysts.

Investigations and management

Investigations—those of particular note include: (1) serum angiotensin converting enzyme (ACE) levels—elevated in 60% of patients with acute and one-third of those with chronic disease; (2) bronchoalveolar lavage—revealing increased lymphocytes, especially an increased CD4:CD8 ratio; (3) transbronchial biopsy—noncaseating granulomas found in greater than 60% of stage 1 and 80% of stage 2 or 3 disease; (4) gallium scan—uptake in the parotid and conjunctiva (the ‘panda’ sign) and/or in the hilar nodes (the ‘lambda’ sign) are fairly characteristic and useful confirmation of diagnosis in difficult cases; (5) characteristic changes on CT scan of chest or positive emission tomography (PET) scan.

Management—there is no single treatment for all patients with sarcoidosis. Key issues are to determine (1) whether the patient requires treatment, this usually being based on symptoms, and then (2) the extent of symptomatic disease, and (3) whether this is acute or chronic. First line treatment is usually with corticosteroids, often prednisolone 20 to 40 mg/day (initial dosage, followed by gradual reduction) if topical administration is not possible, although it is not universally accepted that steroids change the course of the disease. If the dose of steroid cannot be reduced to an acceptable level, or if the patient is not responding, then other agents (e.g. chloroquine/hydroxychloroquine, methotrexate, leflunomide, infliximab) are added.

Prognosis—most patients with sarcoidosis will experience disease resolution within 2 to 5 years; about 25% will develop residual fibrosis in the lungs or elsewhere; in a few the disease will become chronic and persist for more than 5 years. Most series from referral centres report 5% disease-related mortality, usually from respiratory failure.

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