Granulomatosis with polyangiitis

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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

  • Upper respiratory, pulmonary and renal disease + constitutional symptoms
  • White, older patients
  • Constitutional symptoms: Fever, malaise, weight loss
  • Upper airway: Serous otits media, hearing loss, sinusitis, nasal mucosal ulcerations, septal perforation, epistaxis, laryngotracheal disease
    • Subglottic stenosis MC laryngotracheal lesion (16% patients)
  • Lower respiratory: Cough, dyspnea, pleuritis, hemoptysis, diffuse alveolar hemorrhage
  • Renal failure, glomerulonephritis
  • Ophthalmologic: scleritis, episcleritis, uveitis
  • Cutaneous: Palpable purpura, nodules, ulcers
  • Neurologic: Mononeuropathy and polyneuropathy, cerebral vasculitis, cerebral hemorrhage or thrombosis
  • Cardiac: Pericarditis, myocarditis

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, normochronic 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:
    • Methylprednisolone (7-15mg/kg/d with max 1000mg)
    • Prednisone 1mg/kg/d (max 80mg)
  • 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