title: Successful management of Churg-Strauss syndrome using omalizumab as adjuvant immunomodulatory therapy: first documented pediatric case.
abstract: Churg-Strauss syndrome (CSS) is an anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitis; it is extremely rare in childhood and defined according to the Chapel-Hill Consensus as an eosinophil-rich and granulomatous inflammation involving the respiratory tract and necrotizing vasculitis affecting small to medium-sized vessels. Children commonly have a history of asthma and sinusitis whilst clinical presentation typically involves pulmonary tract and less frequently skin, heart, gastrointestinal tract, and peripheral nerves. Cardiopulmonary disease is higher in children and prognosis is worse. It is associated with significant eosinophilia and raised serum IgE-levels. ANCA are only found in 25% of childhood cases. Here we report the case of a 10-year-old girl who presented to us with vomiting, abdominal pain, and weight loss, paresthesias of lower extremities and breathlessness as well as a history of asthma, sinusitis and allergic rhinitis. She was treated with corticosteroids, cyclophosphamide, intravenous immunoglobulin, mycophenolate mofetil (MMF), and rituximab. However, remission was only achieved after initiation of omalizumab therapy, a recombinant humanized anti-IgE antibody. To the best of our knowledge this is the first pediatric patient suffering from CSS successfully managed with adjuvant anti-IgE therapy resulting in the control of respiratory as well as gastrointestinal symptoms.
authors.affiliation: Department of Paediatric Infectious Diseases and Immunology, Hospital Universitario Virgen del Rocío (HUVR), Sevilla, Spain; Unit of Pediatric Rheumatology, Department of Pediatrics, Hospital Sant Joan de Déu, Esplugues de Llobregat, Barcelona, Spain.
authors.forename: E
authors.initials: E
authors.lastname: Iglesias
authors.forename: M
authors.initials: M
authors.lastname: Camacho Lovillo
authors.forename: I
authors.initials: I
authors.lastname: Delgado Pecellín
authors.forename: M J
authors.initials: MJ
authors.lastname: Lirola Cruz
authors.forename: M D
authors.initials: MD
authors.lastname: Falcón Neyra
authors.forename: J C
authors.initials: JC
authors.lastname: Salazar Quero
authors.forename: J
authors.initials: J
authors.lastname: Bernabeu-Wittel
authors.forename: J P
authors.initials: JP
authors.lastname: González Valencia
authors.forename: O
authors.initials: O
authors.lastname: Neth
mesh_terms: D000888:Antibodies, Anti-Idiotypic / Q000627:therapeutic use*; D061067:Antibodies, Monoclonal, Humanized / Q000627:therapeutic use*; D002648:Child; D015267:Churg-Strauss Syndrome / Q000150:complications / Q000175:diagnosis / Q000188:drug therapy*; D005260:Female; D006801:Humans; D056747:Immunomodulation; D008168:Lung / Q000000981:diagnostic imaging*; D000069444:Omalizumab; D010490:Pericardial Effusion / Q000000981:diagnostic imaging* / Q000209:etiology; D011859:Radiography; D012867:Skin / Q000473:pathology*; D016896:Treatment Outcome; D014463:Ultrasonography
chemical_list: D000888:Antibodies, Anti-Idiotypic; D061067:Antibodies, Monoclonal, Humanized; D000069444:Omalizumab
keywords: asthma; children; omalizumab; vasculitis