What is limited granulomatosis with polyangiitis (GPA)?

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

This designation is reserved for cases that fulfill the modified American College of Rheumatology criteria for the classification of GPA in the absence of disease features that pose immediate threats to either a critical individual organ or to the patient's life. Specifically, this means the following:

  • The patient has no RBC casts in the urine

  • If hematuria is present (but no RBC casts), the serum creatinine level is 1.4mg/dL or less, and there must be no evidence of a rise in the creatinine level of more than 25% above the patient’s baseline level

  • Pulmonary involvement must be circumscribed, such that the room air partial pressure of oxygen (PO2) level is greater than 70 mm Hg or the room air O2 saturation by pulse oximetry is greater than 92%; pulmonary hemorrhage may be treated as limited disease provided that there is no evidence of progression of process (in the absence of data on progression, pulmonary hemorrhage may be treated as severe disease at the discretion of the physician)

  • No disease may exist within any other critical organ (eg, the gastrointestinal [GI] tract, eyes, central nervous system [CNS]) that, without the immediate institution of maximal therapy (ie, pulse methylprednisolone or daily oral cyclophosphamide), threatens the function of that organ and/or the patient’s life [9]


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