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doi:10.1378/chest.06-1208
(Chest. 2007; 131:592-594)
© 2007 American College of Chest Physicians
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Familial Churg-Strauss Syndrome in Two Sisters*

Naomi Tsurikisawa, MD; Sonoko Morita, MD; Takahiro Tsuburai, MD; Chiyako Oshikata, MD; Emiko Ono, MD; Masami Taniguchi, MD; Hiroshi Saito, PhD; Yukiyoshi Yanagihara, PhD and Kazuo Akiyama, MD

* From the Clinical Research Center for Allergy and Rheumatology, National Hospital Organization Sagamihara National Hospital, Sagamihara, Kanagawa, Japan.

Correspondence to: Naomi Tsurikisawa, MD, Clinical Research Center for Allergy and Rheumatology, National Hospital Organization Sagamihara National Hospital, 18-1 Sakuradai, Sagamihara, Kanagawa, Japan 228-8522; e-mail: n-tsurikisawa{at}sagamihara-hosp.gr.jp

Churg-Strauss syndrome (CSS) is an uncommon systemic vasculitis with an increase in the number of eosinophils in the peripheral blood and tissues. Its pathogenesis is unknown, and there is no evidence that genetic factors influence susceptibility to this disease. We present a case of familial CSS in two sisters with atopic-type bronchial asthma and negative perinuclear anti-neutrophil cytoplasmic antibody results. We investigated the human leukocyte antigen typing of the sisters and their six living siblings but found no evidence for heritability of CSS. To our knowledge, this is the first report of familial CSS.

Key Words: asthma • Churg-Strauss syndrome • familial case • heredity • human leukocyte antigen






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