title: Clinical presentation of Churg-Strauss syndrome in children: A 12-year-old-boy with ANCA-negative Churg-Strauss syndrome.
abstract: Churg-Strauss syndrome is an uncommon multisystem disorder characterized by asthma, eosinophilia and vasculitis. We report on a 12-year-old boy with asthma and deterioration of his general condition, who was eventually diagnosed with an ANCA-negative Churg-Strauss syndrome. The propositus included, 50 cases of childhood Churg-Strauss syndrome have been reported. The patient characteristics and clinical characteristics of these children are summarized. The respiratory tract is most frequently involved with pulmonary infiltrates, asthma and sinusitis. Early recognition of childhood Churg-Strauss syndrome is important as delayed diagnosis can lead to severe organ involvement, and possible fatal outcome.
authors.affiliation: Department of Pediatrics, Orbis Medical Center, PO Box 5500, 6130 MB Sittard, The Netherlands.
authors.forename: Femke G E M
authors.initials: FG
authors.lastname: Razenberg
authors.affiliation: Department of Pediatrics, Orbis Medical Center, PO Box 5500, 6130 MB Sittard, The Netherlands.
authors.forename: Jan W C M
authors.initials: JW
authors.lastname: Heynens
authors.affiliation: Department of Pulmonology, Orbis Medical Center, Sittard, The Netherlands.
authors.forename: Geeuwke
authors.initials: G
authors.lastname: Jan de Vries
authors.affiliation: Department of Pediatrics, Division of Respiratory Medicine, Erasmus Medical Center, Rotterdam, The Netherlands ; Department of Epidemiology, Erasmus Medical Center, Rotterdam, The Netherlands.
authors.forename: Liesbeth
authors.initials: L
authors.lastname: Duijts
authors.affiliation: Department of Pediatrics, Division of Respiratory Medicine, Erasmus Medical Center, Rotterdam, The Netherlands.
authors.forename: Johan C
authors.initials: JC
authors.lastname: de Jongste
authors.affiliation: Université Paris Descartes, Assistance Publique des Hôpitaux de Paris, Service de Pneumologie et Allergologie Pédiatriques, Hôpital Necker Enfants Malades, Paris, France.
authors.forename: Jacques
authors.initials: J
authors.lastname: de Blic
authors.affiliation: Department of Pediatrics, Orbis Medical Center, PO Box 5500, 6130 MB Sittard, The Netherlands.
authors.forename: Philippe P R
authors.initials: PP
authors.lastname: Rosias
keywords: ACR, American College of Rheumatology; ANCA, antineutrophil cytoplasmic antibody; Asthma; CCS, Churg–Strauss syndrome; CT, computed tomography; Child; Churg–Strauss syndrome; ELD, eosinophilic lung disease; Eosinophilia; Eosinophilic lung disease; FeNO, fractional exhaled nitric oxide; Ig, immunoglobulin; Pulmonary infiltrate; Vasculitis