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The Epidemiology of Antineutrophil Cytoplasmic Autoantibody-Associated Vasculitis in Olmsted County, Minnesota: A Twenty-Year US Population-Based Study.

Abstract

OBJECTIVE

To estimate the annual incidence, prevalence, and mortality of antineutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis (AAV) and its subsets, granulomatosis with polyangiitis (Wegener's) (GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (Churg-Strauss) (EGPA), in a US-based adult population.

METHODS

All medical records of patients with a diagnosis of, or suspicion of having, AAV in Olmsted County, Minnesota from January 1, 1996 to December 31, 2015 were reviewed. AAV incidence rates were age- and sex-adjusted to the 2010 US white population. Age- and sex-adjusted prevalence of AAV was calculated on January 1, 2015. Survival rates observed in the study cohort were compared with expected rates in the Minnesota population.

RESULTS

Of the 58 incident cases of AAV in Olmsted County during the study period, 23 (40%) were cases of GPA, 28 (48%) were cases of MPA, and 7 (12%) were cases of EGPA. Overall, 28 (48%) of the patients with AAV were women and 57 (98%) were white. The mean ± SD age at diagnosis was 61.1 ± 16.5 years. Thirty-four patients (61%) had myeloperoxidase (MPO)-ANCAs, and 17 (30%) were positive for proteinase 3 (PR3)-ANCAs; 5 (9%) were ANCA-negative. The annual incidence of AAV was 3.3 per 100,000 population (95% confidence interval [95% CI] 2.4-4.1). The incidence rates of GPA, MPA, and EGPA were 1.3 (95% CI 0.8-1.8), 1.6 (95% CI 1.0-2.2), and 0.4 (95% CI 0.1-0.6), respectively. The overall prevalence of AAV was 42.1 per 100,000 (95% CI 29.6-54.6). The mortality rate among AAV patients overall, and among patients with EGPA, those with MPA, and those with MPO-ANCAs, was increased in comparison to the Minnesota general population (each P < 0.05), whereas mortality rates among patients with GPA, those with PR3-ANCAs, and ANCA-negative patients did not differ from that in the general population.

CONCLUSION

The annual incidence of AAV in Olmsted County, Minnesota over the 20 years of the study was 3.3 per 100,000, with a prevalence of 42.1 per 100,000, which is substantially higher than the rates reported in other areas worldwide. The incidence of GPA was similar to that of MPA. Patients with MPA and those with EGPA, but not patients with GPA, experienced higher rates of mortality than that in the Minnesota general population. MPO-ANCAs were a marker of poor survival in this population of patients with AAV.

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  • Authors+Show Affiliations

    ,

    Mayo Clinic College of Medicine and Science, Rochester, Minnesota. San Raffaele Scientific Institute, Milan, Italy. Santa Chiara Hospital, Trento, Italy.

    ,

    Mayo Clinic College of Medicine and Science, Rochester, Minnesota. INSERM UMR1227, Lymphocytes B et Autoimmunité, Université de Bretagne Occidentale, CHU de Brest, Brest, France.

    ,

    Mayo Clinic College of Medicine and Science, Rochester, Minnesota.

    ,

    Mayo Clinic College of Medicine and Science, Rochester, Minnesota.

    Mayo Clinic College of Medicine and Science, Rochester, Minnesota.

    Source

    Arthritis & rheumatology (Hoboken, N.J.) 69:12 2017 12 pg 2338-2350

    MeSH

    Adult
    Aged
    Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis
    Antibodies, Antineutrophil Cytoplasmic
    Churg-Strauss Syndrome
    Cohort Studies
    Female
    Granulomatosis with Polyangiitis
    Humans
    Incidence
    Male
    Microscopic Polyangiitis
    Middle Aged
    Minnesota
    Myeloblastin
    Peroxidase
    Prevalence
    Survival Rate
    Time Factors

    Pub Type(s)

    Journal Article
    Research Support, N.I.H., Extramural

    Language

    eng

    PubMed ID

    28881446

    Citation

    Berti, Alvise, et al. "The Epidemiology of Antineutrophil Cytoplasmic Autoantibody-Associated Vasculitis in Olmsted County, Minnesota: a Twenty-Year US Population-Based Study." Arthritis & Rheumatology (Hoboken, N.J.), vol. 69, no. 12, 2017, pp. 2338-2350.
    Berti A, Cornec D, Crowson CS, et al. The Epidemiology of Antineutrophil Cytoplasmic Autoantibody-Associated Vasculitis in Olmsted County, Minnesota: A Twenty-Year US Population-Based Study. Arthritis & rheumatology (Hoboken, N.J.). 2017;69(12):2338-2350.
    Berti, A., Cornec, D., Crowson, C. S., Specks, U., & Matteson, E. L. (2017). The Epidemiology of Antineutrophil Cytoplasmic Autoantibody-Associated Vasculitis in Olmsted County, Minnesota: A Twenty-Year US Population-Based Study. Arthritis & Rheumatology (Hoboken, N.J.), 69(12), pp. 2338-2350. doi:10.1002/art.40313.
    Berti A, et al. The Epidemiology of Antineutrophil Cytoplasmic Autoantibody-Associated Vasculitis in Olmsted County, Minnesota: a Twenty-Year US Population-Based Study. Arthritis & rheumatology (Hoboken, N.J.). 2017;69(12):2338-2350. PubMed PMID: 28881446.
    * Article titles in AMA citation format should be in sentence-case
    TY - JOUR T1 - The Epidemiology of Antineutrophil Cytoplasmic Autoantibody-Associated Vasculitis in Olmsted County, Minnesota: A Twenty-Year US Population-Based Study. AU - Berti,Alvise, AU - Cornec,Divi, AU - Crowson,Cynthia S, AU - Specks,Ulrich, AU - Matteson,Eric L, Y1 - 2017/11/09/ PY - 2017/04/21/received PY - 2017/08/29/accepted PY - 2017/9/8/pubmed PY - 2017/12/12/medline PY - 2017/9/8/entrez SP - 2338 EP - 2350 JF - Arthritis & rheumatology (Hoboken, N.J.) VL - 69 IS - 12 N2 - OBJECTIVE: To estimate the annual incidence, prevalence, and mortality of antineutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis (AAV) and its subsets, granulomatosis with polyangiitis (Wegener's) (GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (Churg-Strauss) (EGPA), in a US-based adult population. METHODS: All medical records of patients with a diagnosis of, or suspicion of having, AAV in Olmsted County, Minnesota from January 1, 1996 to December 31, 2015 were reviewed. AAV incidence rates were age- and sex-adjusted to the 2010 US white population. Age- and sex-adjusted prevalence of AAV was calculated on January 1, 2015. Survival rates observed in the study cohort were compared with expected rates in the Minnesota population. RESULTS: Of the 58 incident cases of AAV in Olmsted County during the study period, 23 (40%) were cases of GPA, 28 (48%) were cases of MPA, and 7 (12%) were cases of EGPA. Overall, 28 (48%) of the patients with AAV were women and 57 (98%) were white. The mean ± SD age at diagnosis was 61.1 ± 16.5 years. Thirty-four patients (61%) had myeloperoxidase (MPO)-ANCAs, and 17 (30%) were positive for proteinase 3 (PR3)-ANCAs; 5 (9%) were ANCA-negative. The annual incidence of AAV was 3.3 per 100,000 population (95% confidence interval [95% CI] 2.4-4.1). The incidence rates of GPA, MPA, and EGPA were 1.3 (95% CI 0.8-1.8), 1.6 (95% CI 1.0-2.2), and 0.4 (95% CI 0.1-0.6), respectively. The overall prevalence of AAV was 42.1 per 100,000 (95% CI 29.6-54.6). The mortality rate among AAV patients overall, and among patients with EGPA, those with MPA, and those with MPO-ANCAs, was increased in comparison to the Minnesota general population (each P < 0.05), whereas mortality rates among patients with GPA, those with PR3-ANCAs, and ANCA-negative patients did not differ from that in the general population. CONCLUSION: The annual incidence of AAV in Olmsted County, Minnesota over the 20 years of the study was 3.3 per 100,000, with a prevalence of 42.1 per 100,000, which is substantially higher than the rates reported in other areas worldwide. The incidence of GPA was similar to that of MPA. Patients with MPA and those with EGPA, but not patients with GPA, experienced higher rates of mortality than that in the Minnesota general population. MPO-ANCAs were a marker of poor survival in this population of patients with AAV. SN - 2326-5205 UR - https://www.unboundmedicine.com/medline/citation/28881446/The_Epidemiology_of_Antineutrophil_Cytoplasmic_Autoantibody_Associated_Vasculitis_in_Olmsted_County_Minnesota:_A_Twenty_Year_US_Population_Based_Study_ L2 - https://doi.org/10.1002/art.40313 DB - PRIME DP - Unbound Medicine ER -