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Granulomatosis with Polyangiitis in Children

  • David A. Cabral
  • Kimberly Morishita
Chapter

Abstract

Granulomatosis with polyangiitis (GPA) is a chronic systemic vasculitis of small- to medium-sized arteries. Although rare in childhood, the burden on the child, their caregivers, and the healthcare system is high. GPA in children is most commonly an acutely presenting life- or organ-threatening disease with subsequent chronic and relapsing course and ongoing treatment requirements. Early recognition and treatment of GPA is critical to minimizing short- and long-term morbidity and improving outcome. Physicians need to be aware of the clinical scenarios where there needs to be a high index of suspicion for a diagnosis of GPA. It should be considered among the differential diagnoses of children presenting with features that might include prolonged fever, constitutional symptoms, single or multisystem organ involvement and no evidence of infections (e.g. Tuberculosis), while also excluding malignancy, and other autoimmune diseases. Immunosuppressants (cyclophosphamide and corticosteroids) have been lifesaving in the treatment of GPA, but modern reduced dosing strategies and newer therapies aim to limit the potentially damaging side effects of earlier treatments that include serious infections, growth disturbance in the short term, and infertility and malignancy in the long term. While most data on GPA is derived from the adult literature, evidence for its applicability to the growing child is lacking. The need for international pediatric collaborations including registries to study sufficient patient numbers has been recognized and recent efforts are starting to generate pediatric data

Keywords

Granulomatosis with polyangiitis (GPA) Childhood granulomatous vasculitis Vasculitis Childhood Wegener’s granulomatosis Antineutrophil cytoplasmic antibodies (ANCAs) ANCA-associated vasculitis (AAV) 

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© Springer Science+Business Media Singapore 2017

Authors and Affiliations

  1. 1.Division of Rheumatology, Department of PediatricsBritish Columbia Children’s Hospital and University of British ColumbiaVancouverCanada

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