ArticleArthritis & rheumatology (Hoboken, N.J.)2018
Epidemiology and Outcomes of Granulomatosis With Polyangiitis in Pediatric and Working-Age Adult Populations In the United States: Analysis of a Large National Claims Database.
Article in Arthritis & rheumatology (Hoboken, N.J.), 2018. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 51 papers, 1 of them a synthesis that pooled it.
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Who cites it
51 citing papers in PubMed, 1 synthesis or guideline pooled it, 91 citations in OpenAlex.
- Biologic disease modifying antirheumatic drugs and Janus kinase inhibitors in paediatric rheumatology - what we know and what we do not know from randomized controlled trials.Pediatric rheumatology online journal · 2021Pooled it
- From ANCA-mediated vascular injury to coronary microvascular and myocardial involvement in granulomatosis with polyangiitis: an immunocardiology perspective.Rheumatology international · 2026Review
- Uncommon Territory: Multifocal Tumor-like Brain Lesions in Granulomatosis with Polyangiitis.Diagnostics (Basel, Switzerland) · 2026Article
- Kidney Transplantation in Childhood-Onset ANCA-Associated Vasculitis: Long-Term Outcomes and Prognostic Factors.Clinical journal of the American Society of Nephrology : CJASN · 2026Article
- Immune-mediated cochleovestibular dysfunction: clinical spectrum from isolated inner-ear disorders to systemic autoimmune diseases and therapeutic strategies.Frontiers in immunology · 2026Review
- Comprehensive immune profiling identifies alterations in adaptive and innate immune responses in granulomatosis with polyangiitis patients in remission.Frontiers in immunology · 2026Article
- An atypical case of granulomatosis with polyangiitis in a 15-year-old girl presenting with pyoderma gangrenosum-like ulcers on the face.Dermatology online journal · 2025Article
- Strawberry Gingivitis as the Primary Sign of ANCA-Negative Granulomatosis With Polyangiitis (GPA): A Case Report.Clinical case reports · 2025Article
- Trimethoprim sulfamethoxazole prophylaxis and serious infections in granulomatosis with polyangiitis treated with rituximab.Rheumatology (Oxford, England) · 2025Article
- Minimally Invasive Surgical Approach in Granulomatosis with Polyangiitis Complicated with Intramural Descending Aorta Hematoma Followed by Aortic Wall Rupture.Diagnostics (Basel, Switzerland) · 2025Article
- Gastrointestinal bleeding as an early presentation of the pediatric granulomatosis with polyangiitis: A rare report.SAGE open medical case reports · 2025Article
- A Case of Granulomatosis with Polyangiitis Masquerading as Tuberculosis.Journal of Brown hospital medicine · 2025Article
- Pediatric Presentations of Granulomatosis With Polyangiitis: A Double Case Study.Case reports in dermatological medicine · 2025Article
- Respiratory manifestations of pediatric granulomatosis with polyangiitis: A 12-year experience from a tertiary care facility.Pediatric pulmonology · 2025Article
- Article
- A 79-Year-Old Woman With Worsening Headaches and Pachymeningeal Enhancement: A Case Report From the National Multiple Sclerosis Society Case Conference Proceedings.Neurology(R) neuroimmunology & neuroinflammation · 2024Article
- Article
- Impact of Rituximab on Remission Rates in Granulomatosis With Polyangiitis: A Systematic Review.Cureus · 2024Review
- IgG4-related disease-rare but you should not forget it.Advances in rheumatology (London, England) · 2024Review
- A Rare Case of Limited Granulomatosis with Polyangiitis Presenting as Bilateral Parotitis.European journal of case reports in internal medicine · 2024Article
Corrections and comments
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Authors and funding
6 authors at 1 institution in 1 country.
Funding
Abstract
objectiveThe incidence and prevalence of granulomatosis with polyangiitis (GPA) in the US is not well characterized. Owing to its rarity, outcomes data in pediatric-onset GPA are also lacking. The aims of this study were to describe the epidemiology of GPA and outcomes in GPA patients in the US, and to compare outcomes between pediatric and working-age adult patients.
methodsA retrospective cohort study using the 2006-2014 Truven Health Analytics MarketScan Commercial Claims and Encounters Database was conducted. The incidence and prevalence rates of pediatric and adult GPA (age <65 years) were calculated. Outcomes among the 2 age groups were analyzed.
resultsA total of 5,562 cases of GPA were identified, of which 214 (3.8%) were pediatric onset and 5,348 (96.2%) were adult onset. The incidence rate of pediatric-onset GPA was 1.8 cases per 1 million person-years, compared to 12.8 cases per 1 million person-years in working-age adults. There was a slight female preponderance in both groups (63% and 53% among pediatric and adult GPA patients, respectively). Rates of hospitalization and severe infections were high in both children and working-age adults, but children had more frequent hospitalizations (rate ratio [RR] 1.3 [95% confidence interval (95% CI) 1.1-1.4]) and 2-3-times higher rates of leukopenia (RR 2.6 [95% CI 1.5-4.3]), neutropenia (RR 2.2 [95% CI 1.2-4.0]), and hypogammaglobulinemia (RR 3.7 [95% CI 2.0-6.4]). Time-to-event analyses showed no differences in the time to hospitalization, severe infection, major relapse, or end-stage renal disease.
conclusionThis study represents the largest cohort of GPA reported to date. Pediatric GPA patients experienced more frequent hospitalizations and were more vulnerable to hematologic complications than non-elderly adult patients.
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