VasculitisChurg–Strauss Syndrome in Childhood: A Systematic Literature Review and Clinical Comparison with Adult Patients
Section snippets
Literature Search and Data Collection
To identify cases of childhood CSS, we first searched the PubMed database from 1951 to 2007 for the following words: “Churg–Strauss,” “allergic angiitis,” or “allergic granulomatosis” in combination with “childhood,” “children,” “pediatric,” “infancy,” “boy,” or “girl.” Further, we searched abstracts of the American College of Rheumatology (ACR), European League Aganist Rheumatism (EULAR), and American College of Chest Physians (CHEST) American College of Chest Physians meetings from 2003 to
Clinical and Histological Characteristics of Childhood CSS
Our literature search yielded 33 cases of childhood CSS (Table 1). Patients were reported from the following continents: America (n = 15), Europe (n = 9), Asia (n = 8), and Africa (n = 1). The mean age of patients was 12 years (range, 2 to 18 years) and the male-to-female ratio was 0.74. Laboratory evaluation showed significant eosinophilia in all children (mean eosinophil count, 9068 ± 7179 μL; relative eosinophil count, 33 ± 19%). In addition, serum IgE levels were documented in 16 children
Discussion
CSS is a primary systemic vasculitis affecting small- and medium-sized vessels. Asthma and severe eosinophilia in combination with vasculitic organ manifestations are key features in these patients, rendering this disease unique. Although the pathogenesis is still enigmatic (39), it may include the following: (i) activated eosinophils that secrete enzymes directly, causing tissue damage in vascular and perivascular tissues (40, 41), and (ii) chemokines, such as eotaxin-3 (CCL26), which drive
Acknowledgments
This study was supported by the Carmela and Dennis Banfield Honor Grant of the Vasculitis Foundation (to J.Z.). We thank Martin Herrmann for critically reading the manuscript.
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