20- SARCOIDOSIS

• CONNECTIVE TISSUE/AUTOIMMUNE DISEASE–RELATED PULMONARY FIBROSIS

  • Can exclude by testing autoantibodies (antinuclear factor & rheumatoid factor)
• Multisystem granulomatous disease

  Disease of unknown cause affecting multiple organ systems
• Sarcoidosis

  • Most common in northern Europe
  • More severe in Africans
  • Increased incidence among females
  • Increased incidence in patients aged 20-40
• Noncaseating sarcoid granuloma

  Granuloma composed of epithelioid cells, macrophages, and T-cells without caseous necrosis
• Immunopathology of sarcoidosis

  • Decreased cell-mediated reactivity
  • Lymphopenia and low circulating T-cells in blood
  • High CD4 helper cells in bronchoalveolar lavage
• Sarcoidosis presentation

  • 50% asymptomatic (detected on routine chest x-ray)
  • Fatigue, non-productive cough, SOB, fine crepitations O/E
  • Eye manifestations: anterior & posterior uveitis, conjunctival nodules, etc.
  • Skin manifestations: erythema nodosum, maculopapular lesions, lupus pernio, alopecia
  • Can involve any organ system, but pulmonary involvement is most common
• Löfgren syndrome

  Erythema nodosum, arthritis, and hilar adenopathy
• Heerfordt syndrome

  Fever, parotid enlargement, uveitis, and facial palsy
• Differentials for sarcoidosis

  • Lymphoma
  • Pulmonary Tb
  • Bronchial Ca with secondary spread
• Investigations

  • CBC
  • LFT, RFT (abnormal if organ involvement)
  • Hypercalcemia (sarcoid macrophages produce 1,25-dihydroxyvitamin D)
  • Elevated angiotensin-converting enzyme (ACE) (not specific or sensitive)
  • ECG on presentation
  • Chest x-ray
• Chest x-ray

  • For staging
  • Stage 1: Only bilateral hilar lymphadenopathy (BHL)
  • Stage 2: bilateral hilar lymphadenopathy with Pulmonary infiltrates
  • Stage 3: Only Pulmonary infiltrates
  • Stage 4: Pulmonary FIBROSIS
  • Extent on CXR may not correlate with PFT impairment
• Bronchoalveolar lavage

  High helper T-cells (CD4)
• HRCT

  For better assessment and identifying nodes for biopsy
• PFTs

  • Restrictive pattern (↓ TLC, FEV1, FVC, gas transfer)
• Biopsy

  For histological diagnosis (LNs, skin, transbronchial)
• Treatment

  1. Infiltration/abnormal PFT for 6 months after diagnosis

  2. Steroids (prednisolone) 30mg daily for 6 weeks

  3. Steroids 15mg on alternative days for 6-12 months
• Steroids

  Steroid-sparing agents (methotrexate, azathioprine, cyclophosphamide)
• Indications for steroids

  • Infiltration (abnormal PFTs)
  • Hypercalcemia
  • Neurological, myocardial, or ocular involvement
• Causes of death

  • Respiratory failure
  • Renal damage from hypercalcemia
  • Cor pulmonale