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Abstract

Familial vasculitides: Churg-Strauss syndrome and Wegener's granulomatosis in 2 first-degree relatives.

Paolo Manganelli, Roberto Giacosa, Pieranna Fietta, Adele Zanetti and Tauro Maria Neri
The Journal of Rheumatology March 2003, 30 (3) 618-621;
Paolo Manganelli
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Roberto Giacosa
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Pieranna Fietta
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Adele Zanetti
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Tauro Maria Neri
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Abstract

Churg-Strauss syndrome (CSS) and Wegener's granulomatosis (WG) are uncommon primary vasculitides, characterized by the involvement of the small to medium size vessels and by the frequent presence of serum antineutrophil cytoplasmic antibodies (ANCA). The pathogenesis of ANCA associated vasculitides is unclear, but roles for both genetic and environmental factors have been suggested. Familial cases of WG, but not CSS, have been reported. We describe the occurrence of CSS in a man and, 5 years later, WG in his son. These patients live together in an urban area of Northern Italy and share the HLA haplotype A*03; B*07; C*w07; DRB 1*0404, DQB 1*0302. To our knowledge, this is the first report of the familial clustering of CSS and WG in first-degree relatives.

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The Journal of Rheumatology
Vol. 30, Issue 3
1 Mar 2003
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Familial vasculitides: Churg-Strauss syndrome and Wegener's granulomatosis in 2 first-degree relatives.
Paolo Manganelli, Roberto Giacosa, Pieranna Fietta, Adele Zanetti, Tauro Maria Neri
The Journal of Rheumatology Mar 2003, 30 (3) 618-621;

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Familial vasculitides: Churg-Strauss syndrome and Wegener's granulomatosis in 2 first-degree relatives.
Paolo Manganelli, Roberto Giacosa, Pieranna Fietta, Adele Zanetti, Tauro Maria Neri
The Journal of Rheumatology Mar 2003, 30 (3) 618-621;
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