Pulmonology Book

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Wegener's Granulomatosis

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  1. See Also
    1. Interstitial Lung Disease
    2. Small Vessel Vasculitis
    3. Glomerulonephritis
  2. Epidemiology
    1. Peak Incidence during fourth decade of life
  3. Pathophysiology
    1. Classic Triad of Involvement
      1. Interstitial Lung Disease
      2. Glomerulonephritis
      3. Upper respiratory tract (Sinus and nasal disease)
    2. Granulomatous ANCA-Associated Small Vessel Vasculitis
      1. Necrotizing granulomas
  4. Symptoms
    1. Paranasal sinus congestion
      1. Sinus pain
      2. Rhinorrhea
        1. Purulent Nasal discharge
        2. Epistaxis
    2. Respiratory
      1. Cough
      2. Hemoptysis
      3. Dyspnea
  5. Signs
    1. Head and neck changes
      1. Nasal mucosa Ulceration
      2. Septal perforation
      3. Cartilaginous destruction (Saddle nose deformity)
      4. Gingival Ulceration
      5. Sinusitis
      6. Otitis Media
      7. Hearing Loss
    2. Lung Changes
      1. See Interstitial Lung Disease
      2. Pneumonia
    3. Renal
      1. See Glomerulonephritis
    4. Eye Involvement may also occur
      1. Conjunctivitis
      2. Uveitis
      3. Retinitis
      4. Chemosis
      5. Exophthalmos
    5. Rheumatologic
      1. Polyarthritis
  6. Differential Diagnosis
    1. See Interstitial Lung Disease
    2. See ANCA-Associated Small Vessel Vasculitis
    3. Polyarteritis Nodosa
  7. Labs
    1. Antineutrophil Cytoplasmic Antibodies (ANCA)
      1. cANCA positive in 75-90% of patients
      2. pANCA positive in 20% of cases
    2. Complete Blood Count
      1. Anemia
      2. Leukocytosis
      3. Eosinophilia
    3. Urinalysis
      1. Consistent with Glomerulonephritis
    4. Hyperglobulinemia
  8. Radiology: Chest XRay
    1. See Interstitial Lung Disease
    2. Bronchopneumonic patches
    3. Multiple nodular densities (may cavitate)
  9. Diagnostics
    1. Open lung biopsy (most definitive)
    2. Renal and sinus biopsy are often non-diagnostic
  10. Management
    1. Induction Therapy
      1. Cyclophosphamide (Cytoxan)
      2. Corticosteroids
        1. Consider high-dose IV Methylprednisolone for 3 days
    2. Maintenance Therapy
      1. Taper prednisone
      2. Maintain cyclophosphamide for 12 to 18 months
  11. Coarse
    1. Mortality often associated with Renal Failure
  12. References
    1. Allen in Goldman (2000) Cecil Medicine, p. 1529-32
    2. Calabrese in Ruddy (2001) Kelley's Rheum, p. 1167-76

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