Comparing Presenting Clinical Features in 48 Children With Microscopic Polyangiitis to 183 Children Who Have Granulomatosis With Polyangiitis (Wegener's): An ARChiVe Cohort Study

Cabral, David A.; Canter, Debra; Muscal, Eyal; Nanda, Kabita; Wahezi, Dawn M.; Spalding, Steven J.; Twilt, Marinka; Benseler, Susanne M.; Campillo, Sarah; Charuvanij, Sirirat; Dancey, Paul; Eberhard, B. Anne; Elder, Melissa E.; Hersh, Aimee O.; Higgins, Gloria C.; Huber, Adam M.; Khubchandani, Raju; Kim, Susan; Klein-Gitelman, Marisa; Kostik, Mikhail M.; Lawson, Erica; Lee, Tzielan; Lubieniecka, Joanna M.; McCurdy, Deborah; Moorthy, Lakshmi N.; Morishita, Kimberly; Nielsen, Susan; O’Neil, Kathleen M.; Reiff, Andreas; Ristic, Goran; Robinson, Angela Byun; Sarmiento, Angelyne; Shenoi, Susan; Toth, M.; Mater, Heather A. Van; Wagner‐Weiner, Linda; Weiss, Jennifer E.; White, Andrew J.; Yeung, Rae S. M.

doi:10.1002/art.39729

Cited by 112 publications

(138 citation statements)

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“…Pleurisy affected 14% of patients: 12% of patients had respiratory failure and 22% required supplemental oxygen. In 31 patients lung biopsy was performed: in 77% of cases biopsy findings confirmed the presence of vasculitis or were consistent with features of vasculitis [32]. …”

Section: Lung Involvement In Childhood-onset Gpamentioning

confidence: 99%

“…Considering mortality in childhood GPA, together with infections, respiratory complications (including pulmonary haemorrhage and chronic lung disease) were the most common causes of death [25, 32]. …”

Section: Lung Involvement In Childhood-onset Gpamentioning

confidence: 99%

“…Nodular lesions are the most common pulmonary radiologic finding in GPA, in both adults and children [22, 32, 39]. Their presence is not necessarily associated with clinical signs and symptoms.…”

Section: Lung Involvement In Childhood-onset Gpamentioning

confidence: 99%

“…Pulmonary fibrosis is uncommon in patients with antineutrophil cytoplasmic antibodies-associated vasculitis and mainly occurs in microscopic polyangiitis in association with anti-MPO antibodies; nonetheless, it was described in adult patients affected with GPA [48–51] and also in pediatric patients [32]. Interstitial involvement in a treated patient should induce the suspicion of a iatrogenic pneumopathy [39, 52].…”

Section: Lung Involvement In Childhood-onset Gpamentioning

confidence: 99%

“…Sinonasal disease is a common presenting feature of GPA in children, which should be distinguished from infectious or allergic rhinitis or rhinosinusitis [18, 24, 32]. Longstanding disease can damage nasal cartilage, leading to septal perforation and saddle-nose deformity.…”

Section: Introductionmentioning

confidence: 99%

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Lung involvement in childhood onset granulomatosis with polyangiitis

et al. 2017

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Granulomatosis with polyangiitis is an ANCA-associated systemic vasculitis with a low incidence in the pediatric population. Lung involvement is a common manifestation in children affected by granulomatosis with polyangiitis, both at disease’s onset and during flares. Its severity is variable, ranging from asymptomatic pulmonary lesions to dramatic life-threatening clinical presentations such as diffuse alveolar haemorrhage. Several radiologic findings have been described, but the most frequent abnormalities detected are nodular lesions and fixed infiltrates. Interstitial involvement, pleural disease and pulmonary embolism are less common. Histology may show necrotizing or granulomatous vasculitis of small arteries and veins of the lung, but since typical features may be patchy, the site for lung biopsy should be carefully chosen with the help of imaging techniques such as computed tomography. Bronchoalveolar lavage is helpful to confirm the diagnosis of alveolar haemorrhage. Pulmonary function tests are frequently altered, showing a reduction in the diffusion capacity for carbon monoxide, which can be associated with obstructive abnormalities related to airway stenosis. Nodular lung lesions tend to regress with immunosuppressive therapy, but lung disease may also require second line treatments such as plasmapheresis. In cases of massive diffuse alveolar haemorrhage, ventilator support is crucial in the management of the patient.

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Section: Lung Involvement In Childhood-onset Gpamentioning

confidence: 99%

Section: Lung Involvement In Childhood-onset Gpamentioning

confidence: 99%

Section: Lung Involvement In Childhood-onset Gpamentioning

confidence: 99%

Section: Lung Involvement In Childhood-onset Gpamentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Lung involvement in childhood onset granulomatosis with polyangiitis

et al. 2017

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

Morishita

Wagner‐Weiner

Yen

et al. 2022

Arthritis Care & Research

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for the Childhood Arthritis and Rheumatology Research Alliance (CARRA) Antineutrophil Cytoplasmic Antibody-Associated Vasculitis WorkgroupObjective. There is no standardized approach to the treatment of pediatric antineutrophil cytoplasmic antibodyassociated vasculitis (AAV). Because of the rarity of pediatric AAV, randomized trials have not been feasible. The present study of the Childhood Arthritis and Rheumatology Research Alliance (CARRA) was undertaken to establish consensus treatment plans (CTPs) for severe pediatric AAV to enable the future study of comparative effectiveness and safety.Methods. A workgroup of CARRA members (rheumatologists and nephrologists) formed the AAV Workgroup. This group performed a literature review on existing evidence-based treatments and guidelines for the management of AAV. They determined that the target population for CTP development was patients <18 years of age with new-onset granulomatosis with polyangiitis (GPA), microscopic polyangiitis, or renal-limited AAV (eosinophilic GPA was excluded), with presentation confined to those with severe disease (i.e., organ-or life-threatening). Face-to-face consensus conferences employed nominal group techniques to identify treatment strategies for remission induction and remission maintenance, data elements to be systematically collected, and outcomes to be measured over time.Results. The pediatric AAV Workgroup developed 2 CTPs for each of the remission induction and remission maintenance of severe AAV. A glucocorticoid-weaning regimen for induction and maintenance, a core data set, and outcome measures were also defined. A random sample of CARRA membership voted acceptance of the CTPs for remission induction and remission maintenance, with a 94% (75 of 80) and 98% (78 of 80) approval rate, respectively.Conclusion. Consensus methodology established standardized CTPs for treating severe pediatric AAV. These CTPs were in principle accepted by CARRA-wide membership for the evaluation of pragmatic comparative effectiveness in a long-term registry.

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