• Medicine
  • Published in The Journal of rheumatology 2003

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

@article{Manganelli2003FamilialVC,
  title={Familial vasculitides: Churg-Strauss syndrome and Wegener's granulomatosis in 2 first-degree relatives.},
  author={Paolo Manganelli and Roberto Giacosa and Pieranna Fietta and Adele Zanetti and Tauro Maria Neri},
  journal={The Journal of rheumatology},
  year={2003},
  volume={30 3},
  pages={
          618-21
        }
}
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… CONTINUE READING

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