Abstract | OBJECTIVE: METHODS: RESULTS: In total, 105 children with AAV were included in the study. The median age at diagnosis was 13.8 years (interquartile range 10.9-15.8 years). Among the study cohort, 42% of patients achieved remission at 12 months, 49% had inactive disease at postinduction (4-6 months), and 61% had inactive disease at 12 months. The majority of patients improved, even if they did not achieve inactive disease. An improvement in the PVAS score of at least 50% from time of diagnosis to postinduction was seen in 92% of patients. Minor relapses occurred in 12 (24%) of 51 patients after inactive disease had been achieved postinduction. The median PVDI damage score at 12 months was 1 (range 0-6), and 63% of patients had ≥1 PVDI damage item scored as present at 12 months. CONCLUSION: This is the largest study to date to assess disease outcomes in pediatric AAV. Although the study showed that a significant proportion of patients did not achieve remission, the majority of patients responded to treatment. Unfortunately, more than one-half of this patient cohort experienced damage to various organ systems early in their disease course.
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Authors | Kimberly A Morishita, Lakshmi N Moorthy, Joanna M Lubieniecka, Marinka Twilt, Rae S M Yeung, Mary B Toth, Susan Shenoi, Goran Ristic, Susan M Nielsen, Raashid A Luqmani, Suzanne C Li, Tzielan Lee, Erica F Lawson, Mikhail M Kostik, Marisa Klein-Gitelman, Adam M Huber, Aimee O Hersh, Dirk Foell, Melissa E Elder, Barbara A Eberhard, Paul Dancey, Sirirat Charuvanij, Susanne M Benseler, David A Cabral, ARChiVe Investigators Network within the PedVas Initiative |
Journal | Arthritis & rheumatology (Hoboken, N.J.)
(Arthritis Rheumatol)
Vol. 69
Issue 7
Pg. 1470-1479
(07 2017)
ISSN: 2326-5205 [Electronic] United States |
PMID | 28371513
(Publication Type: Journal Article, Research Support, Non-U.S. Gov't)
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Copyright | © 2017, American College of Rheumatology. |
Chemical References |
- Adrenal Cortex Hormones
- Immunosuppressive Agents
- Rituximab
- Cyclophosphamide
- Mycophenolic Acid
- Azathioprine
- Methotrexate
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Topics |
- Adolescent
- Adrenal Cortex Hormones
(therapeutic use)
- Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis
(complications, drug therapy)
- Azathioprine
(therapeutic use)
- Child
- Cohort Studies
- Cyclophosphamide
(therapeutic use)
- Female
- Follow-Up Studies
- Humans
- Immunosuppressive Agents
(therapeutic use)
- Kidney Diseases
(drug therapy, etiology)
- Lung Diseases
(drug therapy, etiology)
- Male
- Methotrexate
(therapeutic use)
- Mycophenolic Acid
(therapeutic use)
- Prospective Studies
- Recurrence
- Registries
- Remission Induction
- Retrospective Studies
- Rituximab
(therapeutic use)
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