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ANCA-Associated Vasculitis (AAV)

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Life Expectancy

Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a heterogeneous group of rare autoimmune diseases in which ANCA target and activate neutrophils. These, in turn, attack the cells lining small blood vessels, causing inflammation and damage.1

Depending on which organ or system is affected, the symptoms of AAV can vary greatly. Clinical features include, but are not limited to, blood and protein in the urine, hypertension, fatigue, rash, hoarseness, numbness and tingling, headaches, muscle weakness, respiratory issues, and gastrointestinal problems, such as nausea, vomiting, and abdominal pain.2 

Although the life expectancy of patients with AAV has increased in recent years, the risk of death in patients with the disease is still higher than that of age- and sex-matched controls.3,4

Factors Affecting Life Expectancy in AAV

Because AAV is multisystemic and can affect various organs and tissues in the body, it can be hard to predict the life expectancy of a patient diagnosed with the disease. However, when major organs and tissues such as the kidneys or the cardiovascular system are affected, life expectancy can be poor.5

Life Expectancy Relative to Disease Subtype

Because ANCA specificity has been shown to be predictive of relapse, a patient’s AAV type can affect their life expectancy. Patients with autoantibodies against PR3 are more than twice as likely to relapse as those who have autoantibodies against the MPO protein. About 75% to 90% of patients with GPA have PR3-ANCA and around 55% of patients with MPA carry autoantibodies against MPO.6,7,8

Read more about AAV types

Life Expectancy Relative to Disease Onset

The age at diagnosis also affects life expectancy, as patients who are older at the time of diagnosis experience a lower life expectancy than those diagnosed at younger ages. A 2018 study conducted in 85 patients with renal biopsy-proven AAV found that a diagnosis age of 58 years or older was associated with shorter patient survival time.9 

Read more about AAV diagnosis

Life Expectancy Relative to Treatment

Treatment with immunosuppressants and steroids affects life expectancy both positively and negatively.5 While the emergent use of immunosuppressant therapy has greatly increased life expectancy for patients with AAV, in some cases, it can also have a detrimental effect. This is because these therapies can increase the risk of infections.5 Long-term corticosteroid use carries cumulative toxicity risk and can also suppress the immune system, further increasing the risk of serious infections.10

Read more about AAV treatment

Life Expectancy Relative to Disease Activity

Other factors significantly associated with mortality in AAV, especially within the first year after diagnosis, include a high comorbidity index, an Eastern Cooperative Oncology Group (ECOG) performance status score of 3 or 4, a lack of cotrimoxazole prophylaxis, and disease activity parameters such as albumin, hemoglobin, and peak serum creatinine levels, dialysis status, and high Five-Factor Score (FFS), which includes age >65 years, cardiac symptoms, gastrointestinal involvement, and serum creatinine levels of 150 μmol/L or higher.11

Read more about AAV complications

Common Causes of Death in Patients With AAV

The main causes of death in patients with AAV in the first year after diagnosis are infections and active vasculitis. The growth of Staphylococcus aureus from the nasopharynx is associated with higher mortality, and patients with a higher Birmingham Vasculitis Activity Score (BVAS) at disease onset have a shorter life expectancy.3

After the first year following diagnosis, the main causes of death in patients with AAV are cardiovascular disease, malignancy, and infections.4

Finally, serious infections due to the use of immunosuppressants to treat AAV can be life threatening and cause death.3

Read more about AAV prognosis

Ways to Increase Life Expectancy in AAV

Having regular check-ups with a physician can increase the life expectancy of a patient with AAV. Since symptoms of the disease can change significantly over time, treatment often needs to be adjusted accordingly, so these visits are of great importance. It is also important to determine whether the symptoms of a patient are caused by the disease or its treatment.4

Many studies have offered ways to reduce mortality in patients with AAV. A 2020 study of 83 patients with AAV aged 65 to 92 years suggests that lowering the dose of corticosteroids and avoiding intravenous methylprednisolone could improve mortality rates.12

A 2018 study involving 149 patients with AAV with renal involvement, aged 68 to 77 years, showed that reducing immunosuppression, detecting infections early, and administering prophylaxis with cotrimoxazole could help reduce mortality rates.11

The results of a phase 3 clinical trial testing the effect of Tavneos® (avacopan) in patients with AAV showed that treatment with Tavneos appeared to be superior to tapering prednisone in terms of sustained remission for up to 52 weeks. During the study, all patients were also treated with cyclophosphamide or Rituxan® (rituximab).13

Read more about AAV clinical trials

References

  1. ANCA vasculitis. UNC Kidney Center. Updated September 2018. Accessed February 22, 2023.
  2. Qasim A, Patel JB. ANCA positive vasculitis. In: StatPearls [Internet]. Treasure Island, FL: StatPearls Publishing; 2022. Updated May 29, 2022. Accessed February 22, 2023.
  3. Dadonienė J, Kumžaitė G, Mačiulytė R, Miltinienė D. Clinical characteristics and long-term survival differences of the ANCA-associated vasculitis group: a cross-sectional study of 27 patients. Acta Med Litu. 2017;24(2):107-112. doi:10.6001/actamedica.v24i2.3491
  4. Flossmann O, Berden A, de Groot K, et al; European Vasculitis Study Group. Long-term patient survival in ANCA-associated vasculitis. Ann Rheum Dis. 2011;70(3):488-494. doi:10.1136/ard.2010.137778
  5. What is the life expectancy of patients with vasculitis? Vasculitis Foundation. September 21, 2012. Accessed February 22, 2023.
  6. Lionaki S, Blyth ER, Hogan SL, et al. Classification of antineutrophil cytoplasmic autoantibody vasculitides: the role of antineutrophil cytoplasmic autoantibody specificity for myeloperoxidase or proteinase 3 in disease recognition and prognosis. Arthritis Rheum. 2012;64(10):3452-3462. doi:10.1002/art.34562
  7. Marco H, Draibe J, Villacorta J, et al; Spanish Group for the Study of Glomerular Disease (GLOSEN). Determinants of renal and patient outcomes in a Spanish cohort of patients with ANCA-associated vasculitis and renal involvement. Clin Rheumatol. 2018;37(4):1065-1074. doi:10.1007/s10067-017-3973-2
  8. Types of ANCA Vasculitis. ANCA Vasculitis News. Updated May 2021. Accessed February 22, 2023.
  9. Salmela A, Törnroth T, Poussa T, Ekstrand A. Prognostic factors for survival and relapse in ANCA-associated vasculitis with renal involvement: a clinical long-term follow-up study. Int J Nephrol. 2018;2018:6369814. doi:10.1155/2018/6369814
  10. Prednisone and other corticosteroids. Mayo Clinic. December 9, 2022. Accessed February 22, 2023. 
  11. Titeca-Beauport D, Francois A, Lobbedez T, et al. Early predictors of one-year mortality in patients over 65 presenting with ANCA-associated renal vasculitis: a retrospective, multicentre study. BMC Nephrol. 2018;19(1):317. doi:10.1186/s12882-018-1102-3
  12. McGovern D, Williams SP, Parsons K, et al. Long-term outcomes in elderly patients with ANCA-associated vasculitis. Rheumatology (Oxford). 2020;59(5):1076-1083. doi:10.1093/rheumatology/kez388
  13. Jayne DRW, Merkel PA, Schall TJ, Bekker P; ADVOCATE Study Group. Avacopan for the treatment of ANCA-associated vasculitis. N Engl J Med. 2021;384(7):599-609. doi:10.1056/NEJMoa2023386

Reviewed by Hasan Avcu, MD, on 2/24/2023.

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