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Clin Rheumatol. 2003 Oct;22(4-5):333-5.

Eosinophilia and hyperimmunoglobulinemia E as the presenting manifestations of Wegener's granulomatosis.

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  • 1Department of Internal Medicine, Division of Rheumatology, Istanbul University, Istanbul Medical Faculty, Istanbul, Turkey.


Eosinophilia has long been known as a hallmark of Churg-Strauss syndrome but has rarely been reported in Wegener's granulomatosis (WG). Here we describe a patient with WG who had skin, kidney and lung involvement as well as striking peripheral eosinophilia and hyperimmunoglobulinaemia E (hyper-IgE). The patient's clinical picture was complicated by intra-alveolar haemorrhage resulting in severe anaemia and respiratory failure. The pulmonary symptoms recovered completely, but the renal involvement evolved into end-stage renal failure despite intensive immunosuppressive treatment, intravenous immunoglobulin and plasmapheresis. We suggest that the presence of eosinophilia and hyper-IgE might contribute to the development of different disease patterns in WG.

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