Prognosis of ANCA vasculitides
What is the prognosis of patients with ANCA vasculitides?
Most patients with pauci-immune glomerulonephritis have a good response to present induction therapy, with nearly 85% achieving remission. With corticosteroids and cyclophosphamide, overall survival rates at 1 and 5 years are 85% and 75%, respectively.
Mortality is significantly greater in older patients, reaching approximately 23% at 1 year for those over the age of 60 and nearly 44% for those over the age of 70. The presence of diffuse alveolar hemorrhage is associated with an 8-fold risk of death, whereas the serum creatinine level at presentation is the major predictor of kidney outcome.
Although pulmonary hemorrhage is a major cause of death in the early weeks after presentation, the major causes of “late” death are infections and cardiovascular disease.
Patients who present with severe kidney failure have a decreased chance of recovering kidney function compared to those with better-preserved GFR. Histopathologic chronicity index score and baseline estimated glomerular filtration rate (greater than or less than 10 mL/min per 1.73 m 2 ) were associated with treatment response by 4 months.
However, there was no identifiable threshold of kidney function at presentation or histopathologic chronicity index score below which treatment could be deemed futile. Among patients who remain dialysis-dependent at 4 months, only 5% subsequently recover kidney function. In the absence of extra-kidney active vasculitis, the risks of continued immunosuppressive therapy beyond 4 months likely outweigh any potential benefit.
Relapsing disease, unfortunately, occurs in almost 50% of patients at 5 years; most respond to therapy but do receive recurrent exposure to immunosuppression and cytotoxic agents. Approximately 20% of patients that survive initial disease presentation eventually will go on to develop end-stage kidney disease (ESKD).
