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Study finds features and treatments for AAV vary across the lifespan

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 antibody-associated vasculitis. – cytoplasmic neutrophils (Abstract #0513).

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

As noted 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 AAV patients.

The retrospective cohort analysis included 1020 patients with GPA and MPA enrolled in the Vasculitis Clinical Research Consortium longitudinal studies from 2013 to 2021. PEG patients (357) were analyzed separately. Participants were divided by age at diagnosis: children (under 18), young adults (18 to 40), middle-aged adults (40 to 65) and elderly (over 65). Children and young adults with EGPA were grouped into one 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 a GPA and to be ANCA/PR3-ANCA positive, while the amount of MPA and MPO-ANCA increased with age, further reflecting the differences in AAV according to age at diagnosis. Symptoms also varied across age groups.

“In general, the children and young adult groups had more ear, nose and throat (particularly nasal septal perforation and subglottic stenosis), gastrointestinal bleeding, alveolar bleeding diffuse and renal manifestations. Older diagnostic groups had more cardiac and nervous system manifestations than younger diagnostic groups and had fewer musculoskeletal, skin, ocular, 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 submitting our abstract, we have drilled down into 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 found a significant difference in the types of drugs used before and after 2012 for the child, young adult and middle-aged groups. This was specifically observed in the proportion of patients using only rituximab. For the EGPA, the only difference was in the middle age group, where the use of cyclophosphamide did not decrease significantly and the use of rituximab only increased.

Dr Bloom says the study did not examine the reason for these differences. But she points out that it’s difficult for pediatric rheumatologists to apply research findings from adult patients to pediatric patients, because children may metabolize drugs differently and have different patterns of organ damage.

The study also found that patients diagnosed after the age of 65 had significantly higher vasculitis damage index (VDI) and ANCA vasculitis damage index (AVID) scores than those diagnosed at the age of 65. ‘childhood.

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

“Since submitting the abstract, we have created what we believe is a more disease-related damage score by combining factors from VDI and AVID that are more specific to disease, rather than aging. applied these analyzes to age cohorts, we no longer saw the statistically significant differences noted in the VDI and AVID analyses,” she 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 the childhood, an important approach given the lack of rigorous research studies involving pediatric patients. [At the same time]there is a need for more research involving children with AAV, which would allow for more evidence-based care and better outcomes.

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


About ACR Convergence

ACR Convergence, the annual meeting of the American College of Rheumatology, is where rheumatology comes together to collaborate, celebrate, come together and learn. With over 320 sessions and thousands of abstracts, it offers a superior combination of basic science, clinical science, business education and interactive discussions 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 dedicated to advancing the specialty of rheumatology that serves nearly 8,500 physicians, healthcare professionals, and scientists worldwide. In doing so, RAC provides education, research, advocacy and practice management support to help its members continue their innovative work and provide quality patient care. Rheumatology professionals are experts in the diagnosis, management and treatment of over 100 different types of arthritis and rheumatic diseases. For more information, visit