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Sarcoidosis

Background knowledge 🧠

Definition

Sarcoidosis is a multi-system granulomatous disorder of unknown aetiology, characterised by the presence of non-caseating granulomas in affected tissues.

Epidemiology

  • Commonly affects adults aged 20-40.
  • Higher prevalence in Afro-Caribbean populations.

Aetiology and Pathophysiology

  • Immune response leads to non-caseating granulomas.
  • Exact cause is unknown but likely involves genetic and environmental factors.

Clinical Features πŸŒ‘️

Clinical Features

  • Many are asymptomatic and discovered incidentally on imaging.
  • Systemic: fever, fatigue, weight loss.
  • Respiratory: dry cough, dyspnoea, chest pain.
  • Eye: uveitis, conjunctivitis, blurred vision.
  • Skin: erythema nodosum, lupus pernio, maculopapular rash.
  • Musculoskeletal: arthralgia, arthritis.

Investigations πŸ§ͺ

Tests

  • Imaging:
    • Chest X-ray: Bilateral hilar lymphadenopathy is classic.
    • HRCT: Nodularity, lymphadenopathy, lung fibrosis.
  • Spirometry: restrictive lung pattern.
  • Blood tests: raised serum ACE levels, hypercalcaemia.
  • Biopsy of affected tissue: non-caseating granulomas.

Management πŸ₯Ό

Management

  • Observation: Many cases resolve spontaneously.
  • Corticosteroids: First-lineΒ therapy for symptomatic sarcoidosis.
  • Other immunosuppressants: Methotrexate, azathioprine if steroids contraindicated or for steroid-sparing effect.
  • Treat specific organ manifestations (e.g., topical steroids for uveitis).

Prognosis

  • Variable, but often good with 2/3 of patients having spontaneous remission.
  • Chronic disease can lead to fibrosisΒ in affected organs.

Complications

  • Chronic respiratory failure.
  • Pulmonary fibrosis.
  • Cardiac arrhythmias or heart failure due to cardiac involvement.
  • Visual impairment from ocular sarcoidosis.

Key Points

  • Sarcoidosis is a multi-system granulomatous disorder characterised by non-caseating granulomas.
  • It is oftenΒ discovered incidentallyΒ on imaging, where bilateral hilar lymphadenopathyΒ is a classic sign.
  • Where patients are symptomatic, these symptoms are systemicΒ and corticosteroids are used first line.
  • Prognosis is often good, with a majority of patients having spontaneous remission.

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Typical features of sarcoidosis include:

Presentation is usually in the third and fourth decades.

Non-caseating granulomas are found.

Erythema nodosum is a feature.

Hypercalcaemia is often found due to increased activation of vitamin D in theΒ lung.

This enzyme is elevated in a wide range of conditions.


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