Study finds AAV characteristics and treatments vary throughout life

Study finds AAV characteristics and treatments vary throughout life

News – ATLANTA — New research presented this week at ACR Convergence 2022, the annual meeting of the American College of Rheumatology, demonstrated an association between age at diagnosis and clinical features and treatments in patients with antineutrophil cytoplasmic antibody-associated vasculitis (Abstract #0513).

Associated with antineutrophil cytoplasmic antibodies vasculitis (AAV) is a group of conditions characterized by the development of autoantibodies against neutrophil proteinase 3 (PR3-ANCA) or myeloperoxidase (MPO-ANCA) proteins, leading to systemic inflammation and damage to small blood vessels. AAV includes granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA).

As stated in the abstract, although clinical features and outcomes often differ between children and adults with the same rheumatic condition, comparative data are limited in AAV. The aim of this study was to examine the relationship between age at diagnosis and disease characteristics, treatments, and damage in patients with AAV.

Retrospective cohort analysis included 1020 GPA and MPA patients enrolled in the Vasculitis Clinical Research Consortium longitudinal studies from 2013 to 2021. EGPA patients (357) were analyzed separately. Participants were divided by age at diagnosis: children (under 18 years of age), young adults (18 to 40 years of age), middle-aged adults (40 to 65 years of age), and older adults (over 65 years of age). . Children and young adults with EGPA were combined into a single category due to small sample size (n = 87).

In the main cohort, 852 patients (84%) had GPA while 165 (16%) had MPA. Most were ANCA positive at diagnosis: 637 (65%) with PR3-ANCA, 247 (25%) with MPO-ANCA, and 9 patients (1%) with both.

More women than men were diagnosed with AAV in all age groups; however, the difference was greatest when diagnosed in childhood and decreased with each progressive age group. Patients diagnosed as children were more likely to have GPA and be ANCA/PR3-ANCA positive, while the amount of MPA and MPO-ANCA increased with age, further reflecting the differences in AAV by age in the study. time of diagnosis. Symptoms also varied between age groups.

“Overall, the children and young adult groups had more ear, nose, and throat (especially nasal septum perforation and subglottic stenosis), gastrointestinal, diffuse alveolar hemorrhage, and renal manifestations. Older diagnosed groups had more cardiac and nervous system manifestations than younger diagnosed groups and experienced fewer musculoskeletal, cutaneous, eye, and sinus manifestations,” says Jessica Bloom, MD, MSCS, pediatric rheumatologist and professor pediatric assistant at Children’s Hospital of Colorado. and the lead author of the study.

More than half of patients diagnosed in childhood received both cyclophosphamide and rituximab, while the percentage of patients receiving cyclophosphamide alone or no drug increased with age at diagnosis.

“Since we submitted our abstract, we have further analyzed the data and split drug use before and after 2012, since rituximab was approved for use in GPA and MPA in 2011,” says Dr. Bloom. “For GPA and MPA, we saw a significant difference in the types of medications used before and after 2012 for the groups of children, young adults, and middle-aged people. This was specifically seen in the proportion of patients using rituximab alone. For EGPA, the only difference was in the middle-aged group, where cyclophosphamide use alone decreased substantially and rituximab use only increased.”

Dr. Bloom says the study didn’t look at the reason for these differences. But she notes that it is challenging for pediatric rheumatologists to apply research results from adult patients to pediatric patients because children may metabolize medications differently and have different patterns of organ involvement.

The study also found that patients diagnosed after age 65 had significantly higher Vasculitis Damage Index (VDI) and ANCA Vasculitis Damage Index (AVID) scores compared with those diagnosed in childhood.

But Dr. Bloom says that some components of the damage scores used to study AAV reflect age-related damage rather than damage from the disease itself.

“Since we submitted the abstract, we have created what we believe to be a more disease-related damage score by combining factors from VDI and AVID that are more specific to disease, rather than aging. After applying these analyzes to the age cohorts, we no longer saw the statistically significant differences seen in the VDI and AVID analyses,” he says.

Overall, Dr. Bloom says the study is “a comprehensive analysis of a North American cohort of ANCA-associated vasculitis that demonstrates the ability to use adult registry data to analyze patients diagnosed in childhood, an important approach given the lack of rigorous research studies involving pediatric patients. [At the same time]There is a need for further research involving children with AAVs, which would allow for more evidence-based care and better outcomes.”

This research was supported by funds from the Vasculitis Clinical Research Consortium, the Vasculitis Foundation, and the National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS).


About RCA convergence

ACR Convergence, the annual meeting of the American College of Rheumatology, is where rheumatology comes together to collaborate, celebrate, congregate and learn. With over 320 sessions and thousands of abstracts, it offers a superior blend of basic science, clinical science, business education, and interactive discussion to improve patient care. For more information about the meeting, visit join the conversation on Twitter by following the official hashtag (#ACR22).

About the American College of Rheumatology

Founded in 1934, the American College of Rheumatology (ACR) is a nonprofit professional association committed to advancing the specialty of rheumatology serving nearly 8,500 physicians, health professionals, and scientists worldwide. In doing so, the ACR provides education, research, advocacy, and practice management support to help its members continue their innovative work and provide quality care to patients. Rheumatology professionals are experts in the diagnosis, management, and treatment of more than 100 different types of arthritis and rheumatic diseases. For more information visit

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