Granulomatosis with polyangiitis

Background

  • Formerly known as Wegener's granulomatosis
  • c-ANCA associated systemic necrotizing vasculitis
  • Small- and medium-sized blood vessels
  • Predilection for upper and lower respiratory tracts and kidneys

Clinical Features

Differential Diagnosis

Evaluation

Classification

  • American College of Rheumatology: 88% sensitivity and 92% specificity for ≥2 criteria
  • Nasal or oral inflammation
  • Abnormal chest radiograph showing nodules, fixed infiltrate, or cavities
  • Abnormal urinary sedimentation (microscopic hematuria)
  • Granulomatous inflammation on biopsy of an artery or perivascular area

Workup

  • Definitive diagnosis: Biopsy
  • ANCA +, RF+
  • CBC: Leukocytosis, normochromic anemia, thrombocytosis
  • ESR/CRP elevated
  • BUN/Cr
  • Urinalysis (hematuria, proteinuria)
  • CXR- Pulmonary infiltrates and nodules
  • CT chest
  • To consider ANA, C3 or C4, cryoglobulins, hepatitis serology, HIV, LFT, blood culture to rule out other pathology
  • Other tests: Bronchoscopy, PFT, sinus CT

Management

  • Priority: Manage pulmonary hemorrhage and renal insufficiency
    • Difficult airway: Diffuse alveolar hemorrhage and subglottic stenosis
      • Fiberoptic intubation through LMA advocated
  • Rheumatology consult + multidisciplinary consults
  • Mild disease: Corticosteroids and methotrexate
    • No active glomerulonephritis or organ-threatening disease
  • Mod-Severe disease: Corticosteroids and cyclophosphamide or rituximab
  • Corticosteroids:
  • Methotrexate: 20-25mg weekly PO or SC
  • Cyclophosphamide: 2mg/kg/d PO or 15mg/kg q2 weeks x3 then q3 weeks
  • Rituximab: 357mg/m² weekly x 4
  • To consider plasma exchange: Severe/rapidly progressive renal disease, concurrent anti-GBM Ab disease, severe pulmonary hemorrhage

Disposition

See Also

References

Authors:

Neil Young