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Paediatric Pulmonology and Allergology
2005 April, Vol. VIII, No. 1 (2914-2922)
CHURG-STRAUSS SYNDROME IN CHILDREN: A PROPOS OF TWO CASES
Debra Boyer*, Sara O.Vargas**, Dubhfeasa Slattery*, Adnrew A.Colin*
Departments of *Medicine and **Pathology of Boston Children's Hospital, Harvard Medical School, Boston, Massachusetts, USA
Churg-Strauss syndrome (CSS) is a vasculitis accompanied by asthma and eosinophilia. It is generally considered a disease of adults; occurrence in children has been reported only 12 times. We report two pediatric patients with CSS manifesting with prominent pulmonary involvement. One a 16-year-old, with a prior history of asthma, presented with pleuritic chest pain and a peripheral pulmonary nodule complicated by a pleural effusion. The other presented at age 6 with cough, weight loss, and radiographic infiltrates. Lung biopsies revealed elements characteristic of CSS including eosinophilic microabscesses and vasculitis. Five- and three-year follow-up showed continued symptoms in both patients despite medical therapy. Both patients illustrate many of the typical features of CSS. We report these cases to expand the scant knowledge about pediatric CSS and to heighten awareness that this serious disease may affect the pediatric population. The relevant literature on CSS is reviewed with specific reference to childhood cases.