Published online August 7, 2006
PEDIATRICS Vol. 118 No. 3 September 2006, pp. e914-e920 (doi:10.1542/10.1542/peds.2006-0113)
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EXPERIENCE & REASON

Churg-Strauss Syndrome in Children: A Clinical and Pathologic Review

Debra Boyer, MDa, Sara O. Vargas, MDb, Dubhfeasa Slattery, MDa, Yadira M. Rivera-Sanchez, MDa and Andrew A. Colin, MDa

a Departments of Medicine (Division of Respiratory Diseases)
b Pathology, Children's Hospital Boston, Harvard Medical School, Boston, Massachusetts

ABSTRACT

Churg-Strauss syndrome is a vasculitis accompanied by asthma and eosinophilia. It is generally considered a disease of adults; occurrence in children has been reported infrequently. Here we report 2 pediatric patients with Churg-Strauss syndrome manifesting with prominent pulmonary involvement. One, a 16-year-old with a previous history of asthma, presented with pleuritic chest pain and a peripheral pulmonary nodule complicated by an eosinophilic pleural effusion. The other patient presented at age 6 with cough, weight loss, and radiographic infiltrates. Lung biopsies revealed elements characteristic of Churg-Strauss syndrome, including eosinophilic microabscesses and vasculitis. Three- and 5-year follow-up showed continued symptoms in both patients despite medical therapy. Both patients illustrate many of the typical features of Churg-Strauss syndrome. We report these cases to expand the scant knowledge about Churg-Strauss syndrome in pediatric patients and to heighten awareness that this serious disease may affect the pediatric population. The relevant literature on Churg-Strauss syndrome, with specific reference to childhood cases, is reviewed.

Key Words: Churg-Strauss syndrome • pediatric • eosinophilia • pulmonary

Abbreviations: CSS, Churg-Strauss syndrome • CT, computed tomography • WBC, white blood cell • Ig, immunoglobulin • ANCA, antineutrophil cytoplasmic antibodies • p-ANCA, antineutrophil cytoplasmic antibodies with a perinuclear pattern • c-ANCA, antineutrophil cytoplasmic antibodies with a cytoplasmic pattern • ACR, American College of Rheumatology

Accepted Mar 20, 2006.


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Monteleukast ?
Jayendra R Gohil
Pediatrics Online, 18 Aug 2006 [Full text]