What is the prognosis of 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

The remission rate in GPA ranges from 30-93%, depending on the definition of remission and the remission induction therapy used. [34] With aggressive therapy for active disease, more than 50% of patients with GPA recover renal function and are able to become dialysis independent. [35, 36]

Unfortunately, relapse is common in GPA. Typically, up to half of patients with GPA experience relapse within 5 years. [37] The rate (18-40% at 24 mo) and time to first relapse (15-29 mo) varies. [34] Factors associated with relapse include treatment (< 10 g of cyclophosphamide in the first 6 mo, maintaining a high dose of prednisone [>20 mg/day] for < 2.75 mo, and goal of 0 dose of glucocorticoids), ANCA status at diagnosis, and target organ involvement (lung involvement, cardiac involvement, renal involvement, chronic nasal carriage of S aureus). [34, 38]

Rising PR3-ANCA (C-ANCA) titers may correlate with disease activity in approximately two-thirds of patients. However, the relationship is unreliable; thus, negative PR3-ANCA results do not necessarily exclude the possibility of relapse. [37] As significant adverse effects are associated with immunosuppressive therapy, especially cyclophosphamide, ANCA persistence or reappearance should be used as a warning signal rather than an indication to escalate therapy.

Poorer survival is associated with older age, target organ involvement, and target organ damage. Renal involvement has been consistently shown to confer a poorer prognosis. An absence of renal involvement is associated with a 100% 5-year survival rate, compared with approximately 70% in individuals with renal disease. [39] An increased risk of cardiovascular events is also noted. Overall, the 10-year survival rate ranges from 75-88%. [39] Most morbidity in GPA is currently treatment related.


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