Granulomatosis with Polyangiitis

Aka: Granulomatosis with Polyangiitis, Wegener's Granulomatosis

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II. Epidemiology

  1. Rare Incidence
  2. Peak Incidence during fourth decade of life

III. Pathophysiology

  1. Renamed in 2012 as Granulomatosis with Polyangiitis
    1. Previously known as Wegener's Granulomatosis
  2. Classic Triad of Involvement
    1. Interstitial Lung Disease
    2. Glomerulonephritis
    3. Upper respiratory tract (Sinus and nasal disease)
  3. Granulomatous ANCA-Associated Small Vessel Vasculitis
    1. Necrotizing Granulomas

IV. Symptoms

  1. Paranasal Sinus congestion
    1. Sinus pain
    2. Rhinorrhea
      1. Purulent Nasal Discharge
      2. Epistaxis
  2. Respiratory
    1. Cough
    2. Hemoptysis
    3. Dyspnea

V. Signs

  1. Head and neck changes
    1. Nasal mucosa ulceration
    2. Septal perforation
    3. Cartilaginous destruction (Saddle nose deformity)
    4. Gingival ulceration
    5. Recurrent 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. Neurologic
    1. Neuropathy

VI. Differential Diagnosis

  1. See Interstitial Lung Disease
  2. See ANCA-Associated Small Vessel Vasculitis
  3. Polyarteritis Nodosa

VII. Labs

  1. Antineutrophil Cytoplasmic Antibodies (ANCA)
    1. cANCA positive in 75-90% of patients
    2. pANCA positive in 20% of cases
    3. ANCA Test Specificity: 98%
      1. Despite Specificity, high False Positive Rate due to rare Incidence of condition
    4. Indications for ANCA testing (do not obtain solely due to recurrent Sinusitis)
      1. Pulmonary-Renal Syndrome
      2. Rapidly progressive Renal Failure
      3. Mononeuritis multiplex
      4. Pulmonary Hemorrhage
  2. Complete Blood Count
    1. Anemia
    2. Leukocytosis
    3. Eosinophilia
  3. Urinalysis
    1. Consistent with Glomerulonephritis
  4. Hyperglobulinemia

VIII. Imaging: Chest XRay

  1. See Interstitial Lung Disease
  2. Bronchopneumonic patches
  3. Multiple nodular densities (may cavitate)

IX. Diagnostics

  1. Open lung biopsy (most definitive)
  2. Renal and sinus biopsy are often non-diagnostic

X. 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

XI. Coarse

  1. Mortality often associated with Renal Failure

XII. References

  1. Allen in Goldman (2000) Cecil Medicine, p. 1529-32
  2. Calabrese in Ruddy (2001) Kelley's Rheum, p. 1167-76
  3. Ali (2018) Am Fam Physician 98(3): 164-70 [PubMed]

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