Authors: 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
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.
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My comments: It has only been recently that there have been reports of familial Wegener’s Granulomatosis and now the first case of familial CSS. A few patients including myself point to industrial chemicals, etc for this increase in vasculitis disease. At this time, there is no known trigger for these diseases.