Consensus Treatment Plans for Severe Pediatric Antineutrophil Cytoplasmic Antibody–Associated Vasculitis

Morishita, Kimberly; Wagner‐Weiner, Linda; Yen, Eric Y; Sivaraman, Vidya; James, Karen; Gerstbacher, Dana; Szymanski, Ann Marie; O’Neil, Kathleen M.; Cabral, David A; Workgroup, Carra ANCA-Associated Vasculitis

doi:10.1002/acr.24590

Cited by 23 publications

(11 citation statements)

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“…All four patients were treated with glucocorticoids which are the standard of care for chronic vasculitis in children [34,35]. Three patients received rituximab therapy [27,28] and one patient received plasmapheresis [29]; for maintenance therapy, two were treated with cyclophosphamide [28,29], one was treated with mycophenolate mofetil, and one did not continue therapy [27].…”

Section: Results From the Literature Reviewmentioning

confidence: 99%

“…Cyclophosphamide is often added for patients with more severe disease [37]. Other immunosuppressive medications, such as methotrexate and azathioprine, as well as biologic agents (e.g., TNF-inhibitors, rituximab, and tocilizumab) have been used to treat adults with AAV and are increasingly being used to treat chronic vasculitis in the pediatric population [35,38].…”

Section: Results From the Literature Reviewmentioning

confidence: 99%

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A case of ANCA-associated vasculitis in a 16-year-old female following SARS-COV-2 infection and a systematic review of the literature

2022

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Background Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a rare form of vasculitis in children. SARS-CoV-2, the virus that causes COVID-19 infection, seems to trigger autoimmunity and new-onset autoimmune disease in pediatric and adult patients. We present a case of new-onset AAV following COVID-19 infection in an adolescent patient, and we review the literature of AAV following COVID-19 infection. Case presentation An adolescent female with a history of asthma was diagnosed with mild COVID-19 infection and subsequently developed persistent cough, wheezing, hearing loss, arthralgias, and rash. Her imaging and laboratory workup showed pulmonary nodules and cavitary lesions, elevated inflammatory markers, negative infectious testing, and positive ANCA. She was treated with glucocorticoids, rituximab, and mycophenolate mofetil. At six-month follow-up, she had improvement in her symptoms, pulmonary function tests, imaging findings, and laboratory markers. Conclusions We report the second case of new-onset anti-PR3, C-ANCA vasculitis and the fourth case of pediatric-onset AAV following COVID-19 infection. A systematic review of the literature found 6 cases of new-onset AAV in adults after COVID-19 infection. Pediatric and adult patients who develop AAV post COVID-19 infection have few, if any, comorbidities, and show marked radiographic and symptomatic improvement after treatment. There is increasing evidence for COVID-19-induced autoimmunity in children and our case highlights the importance of considering AAV in a child following a recent COVID-19 infection because timely treatment may improve clinical outcomes.

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Section: Results From the Literature Reviewmentioning

confidence: 99%

Section: Results From the Literature Reviewmentioning

confidence: 99%

A case of ANCA-associated vasculitis in a 16-year-old female following SARS-COV-2 infection and a systematic review of the literature

2022

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“…Treatment of pediatric AAV largely relies on data extrapolated from adult experience and randomized control trials, even though long term effects of these medications on a developing immune system continue to be studied (35). All patients in our study received high pulse dose corticosteroids at diagnosis.…”

Section: Discussionmentioning

confidence: 99%

Childhood-Onset ANCA- Associated Vasculitis: Single center experience from Central California

Singh¹,

Sukumaran

2023

Preprint

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Background Childhood-onset ANCA-associated vasculitides (AAV) are rare and characterized by necrotizing inflammation and include granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA). Pediatric data is scare and there have been no prior studies examining the characteristics of pediatric AAV in Central California, which has a Hispanic majority population.Methods We conducted a retrospective study of patients ≤18 years of age diagnosed with AAV between 2010 and 2021, in Central California. We analyzed demographic, clinical and laboratory characteristics, treatment, and initial outcomes.Results Of the 21 patients with AAV, 12 were categorized as MPA. Median age at diagnosis was 13.7 years in MPA cohort and 14 years in GPA. MPA cohort had significantly high proportion of females (92% versus 44%). Fifty seven percent of the cohort were racial/ethnic minority including Hispanics (n = 9), Asians (n = 2), and multiracial (n = 1) and 43% were white (n = 9). Hispanic patients were more frequently diagnosed with MPA (89%) and positive for myeloperoxidase antibody (100%). White patients more often were diagnosed with GPA (78%) and expressed PR-3 positivity (89%). Median duration of symptoms prior to diagnosis was 14 days in MPA cohort and 21 days in GPA cohort. Renal involvement was frequent (100% in MPA and 78% in GPA). GPA cohort had more frequent ENT involvement (89%). MPA cohort tended towards more severe disease with 67% requiring ICU admission and 50% requiring dialysis. Two deaths were reported in MPA cohort, related to Aspergillus pneumonia and pulmonary hemorrhage. In MPA cohort, 42% received cyclophosphamide in combination with steroids and 42% received Rituximab in combination with steroids. GPA patients received cyclophosphamide, either with steroids alone (78%) or in combination with steroids and Rituximab (22%).Conclusions Microscopic polyangiitis was the most frequent AAV subtype with female preponderance, shorter duration of symptoms at onset and higher proportion of racial/ ethnic minority patients. We observed more frequent MPO positivity in Hispanic children. Trends towards higher rates of ICU admission and dialysis was noted in MPA. Patients with MPA received Rituximab more frequently. Future prospective studies are needed to understand differences in childhood onset AAV between diverse racial-ethnic groups.

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“…Treatment should be promptly initiated in case of patients with severe MPA and organ- or potentially life-threatening disease. Based on the most recent European League Against Rheumatism (EULAR) ( 20 ) and Childhood Arthritis and Rheumatology Research Alliance (CARRA) recommendations ( 21 ), the overall treatment plan can be divided into two stages: induction remission and maintenance therapy. In terms of induction remission therapy, intravenous administration of CYC combined with MP is consistently recommended by all guidelines for at least 3 to a maximum of 6 months, which was followed in the present case.…”

Section: Discussionmentioning

confidence: 99%

Atypical Neurological Manifestation in Childhood Microscopic Polyangiitis: A Case Report and Review of Literature

et al. 2022

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Microscopic polyangiitis (MPA), a systemic necrotizing vasculitis of small vessels, is primarily associated with necrotizing and crescentic glomerulonephritis and pulmonary capillaritis. Neurological involvement, particularly of the central nervous system (CNS) is scarcely observed. The diversity of CNS symptoms could puzzle the diagnosis causing delays in treatment and potentially having a considerable effect on patient's quality of life or even death. The aim of this case report is to highlight the unusual manifestation of MPA in order to raise awareness of this orphaned disease among pediatricians or even pediatric rheumatologists and neurologists.Case ReportHerein we report the case of a 13-year-old Thai girl diagnosed with MPA presented with rapidly progressive glomerulonephritis (RPGN). Renal biopsy was performed demonstrated crescentic glomerulonephritis with negative immunofluorescence and positive titer of myeloperoxidase (MPO) antibody. Pulse methylprednisolone (MP) and cyclophosphamide (CYC) as well as plasmapheresis were initiated. Despite treatment with prednisolone (45 mg/day) and monthly CYC for two doses, she experienced a brief generalized tonic–clonic seizure during the follow-up period. The potential differential diagnosis of new-onset neurological manifestation contains infection owing to the immunocompromised status of the patient and CNS vasculitis as a result of the disease itself. Lumbar puncture was performed, and cerebrospinal fluid analysis demonstrated pleocytosis with negative infectious panel. Contrast magnetic resonance imaging studies of the brain showed multifocal patchy T2/FLAIR-hyperintense lesions in the cerebral as well as cerebellum regions, and irregular narrowing along the V4 segment of the right vertebral artery was demonstrated in magnetic resonance angiography. In the presence of CNS vasculitis, pulse MP and CYC were provided. The symptom of nervous system has progressively improved.ConclusionIn our case, MPA revealed RPGN with neurological manifestation. Despite the fact that it is scarcely reported, CNS vasculitis is one of the organ-threatening symptoms. To improve patient morbidity and mortality, multidisciplinary care teams with prompt diagnosis and treatment are highly recommended.

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Consensus Treatment Plans for Severe Pediatric Antineutrophil Cytoplasmic Antibody–Associated Vasculitis

Cited by 23 publications

References 49 publications

A case of ANCA-associated vasculitis in a 16-year-old female following SARS-COV-2 infection and a systematic review of the literature

A case of ANCA-associated vasculitis in a 16-year-old female following SARS-COV-2 infection and a systematic review of the literature

Childhood-Onset ANCA- Associated Vasculitis: Single center experience from Central California

Atypical Neurological Manifestation in Childhood Microscopic Polyangiitis: A Case Report and Review of Literature

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