How is granulomatosis with polyangiitis (GPA) graded?

Updated: Oct 09, 2019
  • Author: Christopher L Tracy, MD; Chief Editor: Herbert S Diamond, MD  more...
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Answer

Current treatment recommendations in GPA depend on the severity and activity of disease. The European Vasculitis Study Group recommends grading disease severity of antineutrophil cytoplasmic antibody (ANCA) – associated vasculitis (AAV) into the following 5 categories [4] :

  • Localized - Upper and/or lower respiratory tract disease without any other systemic involvement or constitutional symptoms

  • Early systemic - Any, without organ-threatening or life-threatening disease

  • Generalized - Renal or other organ-threatening disease, serum creatinine level less than 5.6 mg/dL

  • Severe - Renal or other vital-organ failure, serum creatinine level exceeding 5.6 mg/dL

  • Refractory - Progressive disease unresponsive to glucocorticoids and cyclophosphamide


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