Show Summary Details
Page of

Sarcoidosis 

Sarcoidosis
Chapter:
Sarcoidosis
DOI:
10.1093/med/9780199202447.003.0035
Page of

PRINTED FROM OXFORD MEDICINE ONLINE (www.oxfordmedicine.com). © Oxford University Press, 2016. All Rights Reserved. Under the terms of the licence agreement, an individual user may print out a PDF of a single chapter of a title in Oxford Medicine Online for personal use (for details see Privacy Policy and Legal Notice).

Subscriber: null; date: 16 October 2019

Sarcoidosis Sarcoidosis

Sarcoidosis is a multisystem inflammatory disorder of unknown cause. It is a relatively rare condition (UK incidence 5–10 in 100 000) and so it is unusual for it to present in the acute medical setting.

Acute sarcoid, previously undiagnosed, may present as Löfgren's syndrome, a combination of erythema nodosum, bilateral hilar lymphadenopathy on the CXR, fever, and arthralgia. This requires no specific treatment (other than simple analgesics or NSAIDs for arthralgia/pain from erythema nodosum) and has an excellent prognosis; the majority will resolve completely within 1–2 years.

In the above situation it is worth checking baseline renal function, calcium, and ACE level (which may be raised in sarcoid, although elevated levels are not diagnostic) and referring for outpatient HRCT/PFT and chest clinic follow up to clarify degree of lymphadenopathy and lack of parenchymal involvement. In this situation biopsy of lymph nodes by mediastinoscopy (to exclude lymphoma/TB) is usually not needed unless there is anything atypical about the situation.

Known diagnosis of sarcoid

Respiratory presentation

Causes of increased shortness of breath, on a background of sarcoid, are most commonly due to the following.

Flare of sarcoid

Comparison of CXR with earlier films is important. Both sarcoid and infection can cause an increase in shadowing on the CXR and discrimination between the two can be difficult, although dense consolidation (especially lobar) is unlikely to be sarcoid. Discuss with chest team about need for starting/increasing existing dose of steroid and arranging appropriate outpatient investigation/review. In treating active disease standard treatment would be prednisolone 40 mg od for 4 weeks before tapering to a maintenance dose, ideally under review of a respiratory physician.

Infection

Treat as community-acquired infection. If the patient is on maintenance steroids, consider a transient increase in dose whilst unwell.

Pneumothorax

Occurs in cases of sarcoid with pulmonary fibrosis: treat as secondary pneumothorax.

Non-respiratory presentation

  • Hypercalcaemia.

  • Skin: erythema nodosum (symptomatic treatment and NSAIDs), lupus pernio, and cutaneous sarcoid.

  • Cardiac sarcoidosis: most commonly presents with conduction defects. Therefore perform ECG on all sarcoid patients.

  • Neurosarcoidosis: can present with any neurological symptom. Most common presentation is lower motor neuron facial palsy.

  • Ocular sarcoidosis (sight-threatening): usually anterior uveitis. However episcleritis, scleritis, conjunctivitis, glaucoma, and retinal involvement can occur. Require slit-lamp examination. Refer to ophthalmology.

  • Renal sarcoidosis: rarely presents with acute renal failure.

Bear in mind that other specialities may well need to be involved in care of patients with extra-pulmonary sarcoidosis. Extra-pulmonary sarcoid is often an indication for high-dose immunosuppressive treatment, particularly the following.

  • Cardiac sarcoidosis.

  • Neurosarcoidosis.

  • Hypercalcaemia.

  • Lupus pernio.

  • Sight-threatening ocular sarcoidosis.

  • Hepatic, splenic, or renal sarcoidosis.