ANCA-Associated Vasculitis (AAV)

Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) comprises a group of rare autoimmune disorders that cause inflammation and destruction of the small blood vessels. These antibodies attack certain cells in the body, including neutrophils.1

The 3 types of ANCA-associated vasculitis are distinguished by the organs and tissues involved, as well as the type of protein each targets. Granulomatosis with polyangiitis (GPA), formerly called Wegener granulomatosis, affects small to medium-size blood vessels in the respiratory tract, kidneys, and other organs.1 Eosinophilic granulomatosis with polyangiitis (EGPA), formerly called Churg-Strauss syndrome, affects small to medium-size blood vessels and is associated with asthma and high levels of eosinophils, a type of white blood cell, in the blood.2 Microscopic polyangiitis (MPA) affects small blood vessels in the kidneys, lungs, and other organs.3

Prognosis of Granulomatosis With Polyangiitis

The mortality rate for patients with GPA is highest in the first year following diagnosis in both adults and children. Approximately 70% of GPA-related deaths occur during this period.4  In a recent study, the most common cause of death after the first year was cardiovascular disease (26%), followed by malignancy (22%), infection (20%), and active vasculitis (8%).5 Outcomes are worse in older patients than in younger patients with GPA, and older age and renal impairment are the most important major risk factors for poor outcomes and mortality.4 

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Effects of Treatment on Prognosis of GPA

The prognosis for patients with GPA has improved with the use of immunosuppressive therapy, but the overall survival and risk of relapse vary depending on the severity and extent of organ involvement. One retrospective study going back 4 decades found that overall 5-year survival rates in patients with a diagnosis from 1996 or later were superior to those of cohorts with a diagnosis previous to 1996.6 

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Clinical Studies on Prognosis of GPA

A large national retrospective cohort study, which used US data from 2006 to 2014, reported a mortality rate of 10% in patients who received adequate treatment involving the use of intensive immunosuppressive regimes, such as Imuran® (azathioprine), Rituxan® (rituximab), methotrexate, glucocorticoids, and cyclophosphamide. This rate increased to 80% in untreated patients. Chronic disease and prolonged immunosuppression were often accompanied by relapse, end-organ damage, infection, persistent low-grade disease, and the need for frequent hospitalization, resulting in a poor quality of life and significant economic burden.4 

The most frequent adverse complications observed in both children and adults with GPA were severe infection and frequent hospitalization. Hematologic complications and hospitalization rates were higher in pediatric patients than in adults, whereas the rates of end-stage renal disease, relapse, and severe infection were comparable in the 2 groups.4 

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Prognosis of Eosinophilic Granulomatosis With Polyangiitis 

The overall mortality rate in EGPA is 13.9%. Cardiac involvement is more common in EGPA than in other systemic necrotizing vasculitides (SNVs) and is the leading cause of death in patients with this form of ANCA-associated vasculitis. Alveolar hemorrhage is a rare complication of EGPA, but when present, it is associated with an increased risk of death, with an odds ratio of 8.64. Renal insufficiency (pulmonary-renal syndrome) is also frequently associated with alveolar hemorrhage and can potentially impact mortality.7 

Various long-term follow-up studies have indicated a good prognosis for EGPA and remission in 67.3% to 91.5% of patients with EGPA with treatment. Relapse is associated with ANCA positivity and occurs in approximately one-quarter of patients.2 

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Five-Factor Score for Prognosis of EGPA

In 1996, a study conducted by the French Vasculitis Study Group established the Five-Factor Score (FFS) as a tool to evaluate the prognosis of patients with SNVs, such as polyarteritis nodosa, EGPA, and MPA. The scale was revised in 2011 and is now commonly used by clinicians to assess disease severity. In some cases, it is used to help determine the appropriate first-line therapy, particularly when indicators of a poor prognosis are present.7 

The revised FFS is based on 4 parameters found to be associated with a higher risk of death and 1 parameter associated with a better outcome. Age older than 65 years, cardiac insufficiency, renal insufficiency (stabilized peak creatinine level of 1.7 mg/dL), and gastrointestinal involvement are associated with an unfavorable outcome, whereas the absence of ear-nose-throat manifestations is associated with a better prognosis.7 

The 1996 FFS reported 5-year mortality rates of 12%, 26%, and 46% for FFS scores of 0, 1, and 2 or higher, respectively. A limitation of the FFA score is that it predictive of mortality only at the time of diagnosis and cannot be used as a patient’s disease evolves over time.7 

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Prognosis of Microscopic Polyangiitis

In MPA, renal involvement is a significant clinical feature; reports suggest that it occurs in 80% to 100% of patients, whose presentation may range from asymptomatic proteinuria or hematuria to end-stage renal disease requiring dialysis.8

Mortality rates in MPA have varied considerably across treatment centers. According to a systematic review, the estimated 1-year survival rate ranges from 77% to 100%. The 5- and 10-year survival rates vary from 46% to 85% and from 60% to 80%, respectively. Reports on cause of death are also inconsistent across cohorts; vasculitis was the leading cause of death in 38% to 50% of patients in 2 studies but accounted for only 8% of cases in another study. In addition, between 17% and 62% of patient deaths were related to immunosuppressive treatment.9

Relapses affect nearly one-third of patients with MPA, with a median time to relapse of 15 to 43 months. The frequency of relapse correlates inversely with the length of treatment. Although ANCA positivity, FFS score, and time to diagnosis were not predictors of relapse, a time to diagnosis of 90 days or longer was associated with a higher mean number of relapses.9

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1. Garlapati P, Qurie A. Granulomatosis with polyangiitis. StatPearls. Updated December 5, 2022. Accessed February 21, 2023.

2. Gioffredi A, Maritati F, Oliva E, Buzio C. Eosinophilic granulomatosis with polyangiitis: an overview. Front Immunol. 2014;5:549. doi:10.3389/fimmu.2014.00549

3. Hashmi MF, Jain V, Tiwari V. Microscopic polyangiitis. StatPearls. Updated November 27, 2022. Accessed February 21, 2023.

4. Panupattanapong S, Stwalley DL, White AJ, Olsen MA, French AR, Hartman ME. Epidemiology and outcomes of granulomatosis with polyangiitis (GPA) in pediatric and working-age adults populations in the United States: analysis of a large national claims database. Arthritis Rheumatol. 2018;70(12):2067-2076. doi:10.1002/art.40577

5. Aydın Tufan M, Tekkarışmaz N. Predictive factors of mortality in granulomatosis with polyangiitis: a single-center study. Arch Rheumatol. 2021;36(3):435-444. doi:10.46497/ArchRheumatol.2021.8594

6. Holle JU, Gross WL, Latza U, et al. Improved outcome in 445 patients with Wegener’s granulomatosis in a German vasculitis center over four decades. Arthritis Rheum. 2011;63(1):257-266. doi:10.1002/art.27763

7. Guillevin L, Pagnoux C, Seror R, et al. The Five-Factor Score revisited: assessment of prognoses of systemic necrotizing vasculitides based on the French Vasculitis Study Group (FVSG) cohort. Medicine. 2011;90(1):19. doi:10.1097/MD.0b013e318205a4c6

8. Gulay DI, Hakan C. Clinical features and outcomes of patients with microscopic polyangiitis: experience of two centers. Signa Vitae. 2021;17(2):168-173. doi:10.22514/sv.20.16.0100

9. Corral-Gudino L, Borao-Cengotita-Bengoa M, del Pino-Montes J, Lerma-Márquez JL. Overall survival, renal survival and relapse in patients with microscopic polyangiitis: a systematic review of current evidence. Rheumatology. 2011;50(8):1414-1423. doi:10.1093/rheumatology/ker112

Reviewed by Debjyoti Talukdar, MD, on 2/27/2023.