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

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; DRB1*0404, DQB1*0302. To our knowledge, this is the first report of the familial clustering of CSS and WG in first-degree relatives. (J Rheumatol 2003;30:618-21)

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At on point, I was told that there was a genetic component to the vasculitis disease family, but vasculitis rarely developed in two members of the same family. Here is a research case that proves that vasculitis can develop in first-degree relatives.

However, vasculitis diseases need a trigger for the disease to develop. As the researchers have found there are at least two sides to this equation.