Granulomatosis with Polyangiitis (GPA)

General Overview

  • Antineutrophil cytoplasmic autoantibody (ANCA)- associated vasculitis
    • Previously known as Wegener's granulomatosis
    • Other ANCA-associated vasculitides include
      • Microscopic polyangiitis
      • Eosinophilic granulomatosis with polyangitis (Churg-strauss)
  • Symptoms
    • Consitutional (fever, malaise, anorexia, weight loss)
    • Eye (eye pain, visual changes, proptosis)
    • ENT manifestations (sinusitis, otitis media, otorrhea/rhinorrhea, oral/nasal ulcers)
    • Respiratory (hoarseness, cough, dyspnea, stridor, wheezing, hemoptysis, plerutic pain)
    • Renal (glomerulonephritis - hematuria)
    • Skin (purpura, necrosis, ulceration, urticaria, livedo reticularis, nodules)
  • Diagnosis
    • Positive ANCA with biopsy (for positive histology) to confirm
  • Other investigations
    • ANA, Anti-GBM, C3, C4, cryoglobulins
    • Hepatitis, HIV, LFT, tuberculosis, blood cultures
    • Serum creatinine (eGFR)
    • UA, urine protein
    • Consider CXR/CT chest
  • Treatment
    • Glucocorticoid WITH
      • Methotrexate or rituximab or cyclophosphamide