Scientific and Practical Journal Global Initiative for Consensus in Pediatrics Management of vasculites associated with anti-neutrophil cytoplasmic antibodies

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Abstract

Vasculites associated with anti-neutrophil cytoplasmic antibodies (ANCA) (further AAV) include granulomatosis with polyangiitis (previously Wegener disease, now GPA ), microscopic polyangiitis (MP), eosinophilic granulomatosis with polyangiitis (EGPA, previously Churg-Strauss syndrome) and diseases of particular organs, such as renal vasculitis. Though rarely, AAV occurs in children and is accompanied by severe manifestations and mortality, especially in late diagnosis of disease. In most cases, AAV are associated with serologically detected ANCA. Antibodies (ANCA) directed against proteinase-3 (PR3) or myeloperoxidase (MPO) of neutrophils are associated with GPA or MP in more than 80% of cases. And vice versa, only 50% of patients with EGPA are ANCA-positive; and if ANCA are present, in 75% of cases they are directed against MPO. The use of regimens with cyclophosphamide (CP) and high doses of corticosteroids (CS) for remission induction in the past 25 years have transferred AAV from the category of lethal to chronically recurrent diseases. Research in reduction of cyclophosphamide toxicity have resulted in development of safer immunosuppressants for supportive therapy, such as methotrexate (MT) or azathioprine (AZA). Later, stratification of AAV by severity has singled out patients with mild and moderate courses of disease, who are treated according to protocols that do not include CP. Since 2011, rituximab, a monoclonal anti-CD-20 antibody, has been introduced as the first-line AAV therapy. The article discusses current tactics of management of ANCA-vasculites in adults and children.

About the authors

Alexandr G. Rumyanstev

Federal Research Center of Pediatric Hematology, Oncology, and Immunology named after Dmitry Rogachev

Author for correspondence.
Email: Alexander.Rumyantsev@fccho-moscow.ru
Russian Federation

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