Sarcoidosis

Multisystem inflammatory disease characterised by tissue infiltration with monocytes, T-lymphocytes, and non-caeseating granulomas.

Epidemiology and Risk Factors

Prevalence: ~5/100,000

Pathophysiology

Aetiology

Unknown, likely to be genetic susceptibility combined with an environmental trigger leading to an inflammatory response.

Clinical Manifestations

Common:

  • Constitutional
    • Malaise
    • Fever
    • Weight loss
  • Pulmonary
    • Cough
    • Dyspnoea

System involvement:

  • Airway
    • Muscosal infiltration in ~5%
  • Respiratory
    Occurs in over 90%.
    • Classified into:
      • Isolated hilar disease
      • Adenopathy and parenchymal involvement
      • Parenchymal involvement without adenopathy
      • Pulmonary fibrosis
    • Parenchymal changes are classically peribronchial and perivascular
  • Cardiac
    • Conduction disease
      • Supraventricular arrhythmias
      • Prolonged QT and ventricular arrhythmias
      • PVCs
    • Cardiomyopathy
      • Failure
  • CNS
    • Eye involvement
      • Chronic anterior uveitis
    • Cranial nerve involvement
  • Renal
    • Acute granulomatous interstitial nephritis
    • Nephrolithiasis and nephrocalcinosis
      Calcium deposition subsequently leading to chronic renal failure.
  • Hepatic
    • Cholestasis
    • Hepatic failure
    • Portal hypertension
  • Haematological
    40%.
    • Anaemia
    • Leukopaenia

Diagnostic Approach and DDx

Investigations

Management

Treatment modifies course:

  • Steroids
    First-line.
  • Alternative immunosuppression
    Used if disease progresses despite steroids, or steroids are not tolerated. Evidence is poor. Options include:
    • Methotrexate
    • Azathioprine
    • Cyclophosphamide
    • Mycophenolate

Medical

Surgical

Anaesthetic Considerations

  • A
    • Airway nodules
  • B
    • Respiratory involvement
  • C
    • Conduction abnormalities
    • Pulmonary hypertension
    • Cardiomyopathy
  • E
    • Steroid use
      • Cushingoid features
  • F
    • Hypercalcaemia

Marginal and Ineffective Therapies

Complications

Prognosis

High variable, including:

  • Spontaneous resolution
    Unlikely if it persists over 5 years.
  • Protraction
    Death:
    • Pulmonary
    • Cardiac
    • Neurological

Key Studies

References

  1. Sanders D, Rowland R, Howell T. Sarcoidosis and anaesthesia. BJA Educ. 2016;16(5):173-177. doi:10.1093/bjaed/mkv037