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

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, LFTs, blood culture to rule out other pathology
  • Other tests: Bronchoscopy, PFT, sinus CT

Management

Disposition

See Also

References