Cryptogenic Fibrosing Alveolitis

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Cryptogenic Fibrosing Alveolitis

Idiopathic Fibrosing Alveolitis, CFA, IFA Idiopathic pulmonary fibrosis

• one form of Diffuse Parenchymal Lung Disease
• Chronic inflammatory disease with lung fibrosis

Presentation

• progressive shortness of breath
• cough

Examination

• Introduction and exposure
• Observation
  • oxygen
  • Cushingoid appearance
    • Steroid skin
  • dyspnoea
  • accessory muscle use
  • tachypnoea
• Hands
  • Clubbing(60%)
• Face 
  • central cyanosis
• Neck
• Chest
  • Inspection
  • Palpation
  • Percussion
  • Auscultation
    • fine inspiratory crackles
    • bases > apex
• Ask to see FEV₁ and FVC measurements

Investigations

• Tests to exclude extrinsic allergic alveolitis and sarcoidosis
• serology for autoantibodies for prognosis
• bronchoalveolar lavage
• lung function tests
  • restrictive defect
• CXR
  • fibrotic shadowing,
  • ground glass shadowing,
  • streaks of fibrosis
• CT scan
• biopsy

Immunological features

• B cell follicles in intersitium
  • can contain immunoglobulin
• 50% patients show antinuclear antibodies and/or rheumatoid factor
• circulating immune complexes occur in 50% of patients
• immune complexes found in lung
• increased neutrophils in bronchoalveolar lavage (BAL) (up to 60%)
• macrophages in BAL are activated, eosinophils can be elevated

Treatment

• steroids
• immunosuppresants

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