Sarcoidosis

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Sarcoidosis

• Multi-system, granulomatous disorder
• derangement of immune responsiveness leading to granulomatous inflammation
• commonly affects
  • young adults
• Chronic inflammation of interstitium

Presentation

• CXR shows bilateral hilar lymphadenopathy +/- fibrosis
• skin lesions
  • Erythema Nodosum
• shortness of breath on exercise
• restrictive defect on lung function tests
• fever
• malaise
• arthralgia
• eye lesions
• lymph nodes
• hypercalcaemia
• CVS
• CNS

Immunological features

• sarcoid granuloma
  • focal accumulations of epithelioid cells
  • macrophages
  • lymphocytes (mainly T-cells)
• depressed T-cell reactivity to tuberculin and other antigens (Candida albicans)
• overall lymphocytopenia
  • T-cells low
  • B cells slightly increased
• no overall defect in immune function

In lung

• Immune granulomata in interstitium
  • in or close to small lymphatics
  • heal by organisation leading to fibrosis
• hyperplasia in hilar lymph nodes
• BAL shows increase in number of cells
• number of alveolar macrophages increased / reduced proportion of total
• raised CD4/CD8 ratio in broncho-alveolar lavage
• raised proportions of lymphocytes in brochoalveolar lavage
• Lymphopaemia in blood
• raised immunoglobulins in blood (not all cases)

Diagnosis

• non-caseating epithelioid granulomas
• more than one organ

Epidemiology

• 19/100,000 in UK
• common in USA, rare in Japan
• more severe in blacks than whites

Clinical Features

• peak incidence 20-30
• female > male
• most common presentation is respiratory symptoms / abnormalities of chest X-ray (50%)
• fatigue / weight loss (5%)
• peripheral lymphadenopathy (5%)
• fever (4%)
• pulmonary infiltration
  • progressive
  • eventually leads to Cor Pulmonale & death

Extra-pulmonary manifestations

• skin lesions
  • lupus pernio
  • Erythema Nodosum
• Ocular (25%)
  • anterior uveitis
  • posterior uveitis
  • Conjunctivitis
  • Keratoconjunctivitis sicca
• metabolic (10%)
  • Hypercalcaemia
    • due to high circulating 1,25-dihydroxy D₃
• CNS (2%)
  • chronic meningoencephalitis
  • spinal cord disease
  • cranial nerve palsies
    • bilateral VIIth
  • polyneuropathy
  • myopathy
• bone and joiny
  • arthralgia (5%)
  • small joint polyarthropathy
  • bone cysts in digits
• hepatosplenomegaly
• cardiac involvement (3%)
  • ventricular dysrhythmias
  • conduction defects
  • cardiomyopathy with congestive Heart Failure

Investigation

• Chest X-ray
  • bilateral hilar lymphadenopathy
    • characteristic feature
    • differential - TB, lymphoma, bronchial carcinoma
  • pulmonary infiltration
    • mottling in mid zones
    • → generalised fine nodular shadows
    • →widespread pulmonary line shadows
• CT
• Bloods
  • FBC
    • mild normochromic normocytic anaemia
  • ESR raised
  • Hypercalcaemi
  • hypergammaglobulinaemia
  • ACE
    • raised in 33-50% of patients
• Lung Function Tests
  • restrictive defect (FEV₁/FVC ratio normal or increased)
• Transbronchial biopsy
• Kvein test
  • not done anymore
  • injection of sterile sarcoid tissue

Treatment

• observations
• corticosteroids

Prognosis

• severe in American Blacks
  • death rates 10%
• fatal in <5%
• remits by two years
  • in 2/3 of patients with hilar lymphadenopathy alone
  • in 1/2 with hilar lymphadenopathy + CXR evidence of pulmonary infiltration
  • in 1/3 patients with CXR evidence of infiltration + no lymphadenopathy

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