ANCA-associated vasculitis in childhood: recent advances

Calatroni, Marta; Oliva, Elena; Gianfreda, Davide; Gregorini, Gina; Allinovi, Marco; Ramirez, Giuseppe A.; Bozzolo, Enrica; Monti, Sara; Bracaglia, Claudia; Marucci, Giulia; Bodria, Monica; Sinico, Renato Alberto; Pieruzzi, Federico; Moroni, Gabriella; Pastore, Serena; Emmi, Giacomo; Esposito, Pasquale; Catanoso, Mariagrazia; Barbano, G; Bonanni, Alice; Vaglio, Augusto

doi:10.1186/s13052-017-0364-x

Cited by 83 publications

(67 citation statements)

References 60 publications

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“…Among the diseases with small vessel involvement, some are associated with anti-neutrophil cytoplasmic antibodies (ANCA). Individuals with MPA and GPA for example predominantly have circulating ANCAs against, respectively, the neutrophil proteins myeloperoxidase (MPO) and proteinase 3 (PR3) ( 2 , 3 ). Clinically, PR3- and MPO-ANCA-associated vasculitis (PR3-AAV and MPO-AAV) are difficult to distinguish, but have some distinguishing features at least in adults; PR3-AAV often involves a greater number of organs and inflammation more frequently triggers necrotic lesions, whereas renal-limited disease occurs more often in patients with MPO-AAV ( 4 ).…”

Section: Introductionmentioning

confidence: 99%

“…Clinically, PR3- and MPO-ANCA-associated vasculitis (PR3-AAV and MPO-AAV) are difficult to distinguish, but have some distinguishing features at least in adults; PR3-AAV often involves a greater number of organs and inflammation more frequently triggers necrotic lesions, whereas renal-limited disease occurs more often in patients with MPO-AAV ( 4 ). Data on AAV in pediatric populations are comparably scarce ( 2 ).…”

Section: Introductionmentioning

confidence: 99%

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S100A12 Serum Levels and PMN Counts Are Elevated in Childhood Systemic Vasculitides Especially Involving Proteinase 3 Specific Anti-neutrophil Cytoplasmic Antibodies

et al. 2018

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Objectives: Chronic primary systemic vasculitidies (CPV) are a collection of rare diseases involving inflammation in blood vessels, often in multiple organs. CPV can affect adults and children and may be life- or organ-threatening. Treatments for adult CPV, although effective, have known severe potential toxicities; safety and efficacy of these drugs in pediatric patients is not fully understood. There is an unmet need for biologic measures to assess the level of disease activity and, in turn, inform treatment choices for stopping, starting, or modifying therapy. This observational study determines if S100 calcium-binding protein A12 (S100A12) and common inflammatory indicators are sensitive markers of disease activity in children and adolescents with CPV that could be used to inform a minimal effective dose of therapy.Methods: Clinical data and sera were collected from 56 participants with CPV at study visits from diagnosis to remission. Serum concentrations of S100A12, C-reactive protein (CRP) and hemoglobin (Hb) as well as whole blood cell counts and erythrocyte sedimentation rate (ESR) were measured. Disease activity was inferred by physician's global assessment (PGA) and the pediatric vasculitis activity score (PVAS).Results: Serum concentrations of standard markers of inflammation (ESR, CRP, Hb, absolute blood neutrophil count), and S100A12 track with clinically assessed disease activity. These measures—particularly neutrophil counts and sera concentrations of S100A12–had the most significant correlation with clinical scores of disease activity in those children with vasculitis that is associated with anti-neutrophil cytoplasmic antibodies (ANCA) against proteinase 3.Conclusions: S100A12 and neutrophil counts should be considered in the assessment of disease activity in children with CPV particularly the most common forms of the disease that involve proteinase 3 ANCA.Key messages:- In children with chronic primary systemic vasculitis (CPV), classical measures of inflammation are not formally considered in scoring of disease activity.- Inflammatory markers—specifically S100A12 and neutrophil count—track preferentially with the most common forms of childhood CPV which affect small to medium sized vessels and involve anti neutrophil cytoplasmic antibodies (ANCA) against proteinase-3.

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Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

S100A12 Serum Levels and PMN Counts Are Elevated in Childhood Systemic Vasculitides Especially Involving Proteinase 3 Specific Anti-neutrophil Cytoplasmic Antibodies

et al. 2018

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“…ANCA-associated vasculitis is generally a rare condition, but the high prevalence in our study may reflect the fact that Oslo University Hospital, Rikshospitalet, has a selected patient population. In common with other studies, the median age of onset in our study was the early teenage years for ANCA-associated vasculitis and Takayasu's arteritis, and early school-age for IgA vasculitis (8,9).…”

Section: Discussionmentioning

confidence: 64%

Nyreaffeksjon ved systemisk vaskulitt hos barn

Augestad¹,

Hammarström²,

Flatø³

et al. 2017

Tidsskriftet

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“…GPA in children is rare, and exhibits some differences from adult cases. [27][28][29][30][31] Renal involvement is less common in childhood cases (53-83%), [24][25][26][27]29 whereas laryngeal/tracheal and bronchial involvement is more common in children (>30%). 25,26,32,33 In a meta-analysis of 14 studies of childhood GPA, most common features included (þ) ANCA (> 90%) and involvement of ears/ nose/throat (ENT) (82%), constitutional (73%), kidneys (65%), and/or lower respiratory tract (61%).…”

Section: Pediatric Age Groupmentioning

confidence: 99%

Granulomatosis with Polyangiitis (Wegener's Granulomatosis): Evolving Concepts in Treatment

Lynch

Derhovanessian

Tazelaar

et al. 2018

Semin Respir Crit Care Med

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Granulomatosis with polyangiitis (GPA), formerly termed Wegener's granulomatosis, is the most common of the pulmonary vasculitides. GPA typically involves the upper respiratory tract, lower respiratory tract (bronchi and lung), and kidney, with varying degrees of disseminated vasculitis. Cardinal histologic features include a necrotizing vasculitis involving small vessels, extensive “geographic” necrosis, and granulomatous inflammation. The spectrum and severity of the disease is heterogeneous, ranging from indolent disease involving only one site to fulminant, multiorgan vasculitis. Circulating antibodies against cytoplasmic components of neutrophils (ANCAs) play a role in the pathogenesis, and often correlate with activity of the disease. Treatment strategies are evolving. Cyclophosphamide (CYC) plus corticosteroids was the mainstay of therapy for generalized, multisystemic GPA since the 1970s. However, within the past decade, rituximab (RTX), a monoclonal antibody directed against B cells, has been shown to be at least as effective (and possibly more effective) as CYC. Furthermore, the use of RTX may reduce the need for maintenance immunosuppression. Optimal therapy for GPA remains controversial, and additional studies are required to determine the role and duration of maintenance therapy following successful induction therapy.

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ANCA-associated vasculitis in childhood: recent advances

Cited by 83 publications

References 60 publications

S100A12 Serum Levels and PMN Counts Are Elevated in Childhood Systemic Vasculitides Especially Involving Proteinase 3 Specific Anti-neutrophil Cytoplasmic Antibodies

S100A12 Serum Levels and PMN Counts Are Elevated in Childhood Systemic Vasculitides Especially Involving Proteinase 3 Specific Anti-neutrophil Cytoplasmic Antibodies

Nyreaffeksjon ved systemisk vaskulitt hos barn

Granulomatosis with Polyangiitis (Wegener's Granulomatosis): Evolving Concepts in Treatment

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