What are the mortality rates 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

Severe, untreated GPA is associated with a very high (>90%) mortality rate. Historically, patients with untreated GPA had a mean survival of 5 months from diagnosis; the mortality rate was 82% at 1 year. The introduction of corticosteroids prolonged the median survival by only 7.5 months.

With the advent of cytotoxic therapy, patient survival in GPA markedly improved. According to a meta-analysis, with current treatments, the 5-year survival rate ranges from 74-79%. [39] The 1-year mortality rate is still high, around 11% (range, 2.2-25%), depending on disease severity and the intensity of treatment. [45] In a study of a US inpatient database, the annual hospitalization rate for patients with a principal diagnosis of GPA increased by 24% from 1993 to 2011, from 5.1 to 6.3 per 1,000,000 population; however, in-hospital deaths durig that period declined 73%, from 9.1% to 2.5%. [46]


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