Vasculitis
Churg–Strauss Syndrome in Childhood: A Systematic Literature Review and Clinical Comparison with Adult Patients

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Objective

To describe the clinical characteristics of children with Churg–Strauss syndrome (CSS) in comparison with adult patients.

Materials and methods

A systematic literature analysis was performed in the Medline database up to November 2007 and in rheumatology and pulmonology meeting scientific abstracts 2003-2007. Articles with reported childhood CSS cases were retrieved; clinical data were recorded. Descriptive statistical analyses and a comparison with 2 published adult CSS cohorts were performed.

Results

Thirty-three cases of childhood CSS were identified. The mean age was 12 years and the male-to-female ratio was 0.74. All patients had significant eosinophilia and asthma. Histological evidence of eosinophilia and/or vasculitis was present in virtually all patients. Antineutrophil cytoplasmic antibodies were found in 25% of children with CSS. Initial treatment was corticosteroid monotherapy in 76% of childhood CSS patients, while 24% received additional immunosuppressive therapy. Another 18% required further immunosuppression at follow-up due to frequent relapses. Six deaths (18%), all related to the underlying disease, occurred after a mean disease duration of 14 months. As compared with adult CSS patients, children had a predominance of cardiopulmonary disease manifestations, a lower rate of peripheral nerve involvement, and higher mortality.

Conclusions

Many aspects of CSS are similar in childhood and adult patients. However, pulmonary and cardiac involvement is predominant in pediatric CSS and mortality is substantial.

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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