The Journal of Allergy and Clinical Immunology
Volume 117, Issue 6 , Pages 1292-1302, June 2006

Approaches to the treatment of hypereosinophilic syndromes: A workshop summary report

  • Amy D. Klion, MD

      Affiliations

    • From the Laboratory of Parasitic Diseases, National Institutes of Health, Bethesda
    • Corresponding Author InformationReprint requests: Amy D. Klion, MD, Bldg 4, Rm 126, National Institutes of Health, 4 Center Dr, Bethesda, MD 20892.
    • ,
  • Bruce S. Bochner, MD

      Affiliations

    • Johns Hopkins Asthma and Allergy Center, Baltimore
    • ,
  • Gerald J. Gleich, MD

      Affiliations

    • Departments of Dermatology and Medicine, University of Utah, Salt Lake City
    • ,
  • Thomas B. Nutman, MD

      Affiliations

    • From the Laboratory of Parasitic Diseases, National Institutes of Health, Bethesda
    • ,
  • Marc E. Rothenberg, MD, PhD

      Affiliations

    • Department of Pediatrics, Cincinnati Children's Hospital Medical Center, Cincinnati
    • ,
  • Hans-Uwe Simon, MD, PhD

      Affiliations

    • Department of Pharmacology, University of Bern
    • ,
  • Michael E. Wechsler, MD

      Affiliations

    • Harvard Medical School, Pulmonary and Critical Care Division, Brigham and Women's Hospital, Boston
    • ,
  • Peter F. Weller, MD

      Affiliations

    • Harvard Medical School, Department of Medicine, Beth Israel Deaconess Medical Center, Boston
    • ,
  • the Hypereosinophilic Syndromes Working Group

      Affiliations

    • The members of the Hypereosinophilic Syndromes Working Group are shown in the Acknowledgments.

Received 19 September 2005; received in revised form 22 February 2006; accepted 24 February 2006. published online 06 May 2006.

Bethesda and Baltimore, Md, Salt Lake City, Utah, Cincinnati, Ohio, Bern, Switzerland, and Boston, Mass

Hypereosinophilic syndromes are a heterogeneous group of uncommon disorders characterized by the presence of marked peripheral blood eosinophilia, tissue eosinophilia, or both, resulting in a wide variety of clinical manifestations. Although corticosteroids are the first-line therapy for many of these disorders, approaches to the treatment of patients who do not tolerate or are unresponsive to corticosteroids are poorly standardized. A multidisciplinary group of 37 clinicians and scientists participated in a workshop held in May 2005 in Bern, Switzerland to discuss current and future approaches to therapy for 3 eosinophil-mediated disorders: hypereosinophilic syndrome, Churg-Strauss syndrome, and eosinophil-associated gastrointestinal disease. The goal of the workshop was to summarize available data regarding treatment of these disorders to identify the most promising therapies and approaches for further study. There was consensus among all of the participants that the identification of markers of disease progression to assess treatment responses is a research priority for all 3 disorders. Furthermore, the need for newer therapeutic strategies and novel drugs, as well as multicenter trials to assess all treatment modalities, was emphasized.

Key words: Eosinophil, hypereosinophilic syndromes, Churg-Strauss syndrome, eosinophil-associated gastrointestinal disease, treatment

Abbreviations used: ANCA, Anti-neutrophil cytoplasmic antibody, CSS, Churg-Strauss syndrome, EE, Eosinophilic esophagitis, EGID, Eosinophil-associated gastrointestinal disease, FIP1L1, Fip1-like 1, HES, Hypereosinophilic syndrome, IHES, Idiopathic hypereosinophilic syndrome, L-HES, Lymphocytic variant hypereosinophilic syndrome, PDGFRA, Platelet-derived growth factor receptor α

 

 The workshop was funded by Office of Rare Diseases Research of the National Institutes of Health, GlaxoSmithKline (GSK), and the American Partnership for Eosinophilic Disorders.Disclosure of potential conflict of interest: B. Bochner has consultant arrangements with GlaxoSmithKline. G. Gleich has consultant arrangements with GlaxoSmithKline, Ception Therapeutics, Eosynos, and Novartis Genentech; owns stock in Ception Therapeutics; is founder of Eosynos; has received grants from GlaxoSmithKline and Novartis Genentech; and is on the speakers' bureau for Novartis Genentech. M. Rothenberg has consultant arrangements with GlaxoSmithKline, Ception Therapeutics, and Cambridge Antibody Technology; owns stock in Ception Therapeutics; has received grants from Cambridge Antibody Technology; is on the speakers' bureau for Merck; and is an Honorarium from Tanox. H. Simon has consultant arrangements with GlaxoSmithKline, Greenford, United Kingdom, and has received grants from the Swiss National Science Foundation. M. Wechsler has consultant arrangements with Novartis Genentech; has received grants from Merck; and is on the speakers' bureau for Novartis Genentech and Merck. The rest of the authors have declared that they have no conflict of interest.

PII: S0091-6749(06)00520-3

doi:10.1016/j.jaci.2006.02.042

The Journal of Allergy and Clinical Immunology
Volume 117, Issue 6 , Pages 1292-1302, June 2006

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